記述言語：日本語   出版者・発行元：日本脳腫瘍病理学会  

researchmap

記述言語：日本語   出版者・発行元：(公社)日本皮膚科学会  

researchmap

記述言語：日本語   出版者・発行元：日本脳腫瘍病理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本小児神経外科学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本小児神経外科学会  

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Myxoid pleomorphic liposarcoma (MPLS) is an extremely rare tumor listed in the fifth edition of the WHO classification (2020). Histologically, it mainly comprises a mixture of myxoid and pleomorphic liposarcoma-like components. Genetically, it lacks FUS/EWSR1::DDIT3 fusion and MDM2 amplification. Herein, we describe an example of MPLS with rhabdoid cells in a 10-year-old girl who presented with a growing mass in the right inguinal region. The specimen from the wide excision measured 68 mm × 55 mm × 43 mm, and a circumscribed and lobulated mass was observed in the subcutaneous tissue. Histologically, oval-to-short, spindle-shaped, proliferating tumor cells with moderate nuclear atypia and mesh-like capillaries against a myxoid background were noted. Adipocytes were observed focally, while rhabdoid cells were observed multifocally. Immunohistochemically, the tumor showed inconsistent reactivity for desmin but was negative for MYOD1, myogenin, MDM2, and CDK4. Fluorescence in situ hybridization revealed no DDIT3 rearrangement. Despite adjuvant chemotherapy, the tumor metastasized to the thoracic cavity 24 months after excision. The metastatic lesions contained abundant lipoblasts rather than rhabdoid cells, and we concluded this tumor was a MPLS. The presence of rhabdoid cells could be a diagnostic pitfall, and recognizing such a variation in histology would help improve diagnostic accuracy.

DOI： 10.1177/10668969241226695

PubMed

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：英語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：(公社)神奈川県医師会  

researchmap

記述言語：日本語   出版者・発行元：(公社)神奈川県医師会  

researchmap

記述言語：日本語   出版者・発行元：医学図書出版(株)  

researchmap

記述言語：日本語   出版者・発行元：医学図書出版(株)  

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND/PURPOSE: There is currently no consensus on the use of endoscopic papillectomy (EP) for early stage duodenal ampullary adenocarcinoma. This study aimed to evaluate the feasibility of EP for patients with early stage duodenal ampullary adenocarcinoma. METHODS: Patients who underwent EP for ampullary adenocarcinomas were investigated. Complete and clinical complete resection rates were evaluated. Clinical complete resection was defined as either complete resection or resection with positive or unknown margins but no cancer in the surgically resected specimen, or no recurrence on endoscopy after at least a 1-year follow-up. RESULTS: Adenocarcinoma developed in 30 patients (carcinoma in situ [Tis]: 21, mucosal tumors [T1a(M)]: 4, tumors in the sphincter of Oddi [T1a(OD)]: 5). The complete resection rate was 60.0% (18/30) (Tis: 66.7% [14/21], T1a[M]: 50.0% [2/4], and T1a[OD]: 40.0% [2/5]). The mean follow-up period was 46.8 months. The recurrence rate for all patients was 6.7% (2/30). The clinical complete resection rates of adenocarcinoma were 89.2% (25/28); rates for Tis, T1a(M), and T1a(OD) were 89.4% (17/19), 100% (4/4), and 80% (4/5), respectively. CONCLUSIONS: EP may potentially achieve clinical complete resection of early stage (Tis and T1a) duodenal ampullary adenocarcinomas.

DOI： 10.1002/jhbp.1398

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

PURPOSE: The 2021 World Health Organization (WHO) classification of central nervous system (CNS) tumors uses an integrated approach involving histopathology and molecular profiling. Since majority of adult malignant brain tumors are gliomas and primary central nervous system lymphomas (PCNSL), rapid differentiation of these diseases is required for therapeutic decisions. Additionally, diffuse gliomas require molecular information on single nucleotide variants (SNV), such as IDH1/2. Here, we report an intraoperative integrated diagnostic (i-ID) system to classify CNS malignant tumors, which updates legacy frozen section (FS) diagnosis through incorporation of a quantitative polymerase chain reaction (qPCR)-based genotyping assay. EXPERIMENTAL DESIGN: FS evaluation, including GFAP and CD20 rapid immunohistochemistry, was performed on adult malignant CNS tumors. PCNSL was diagnosed through positive CD20 and negative GFAP immunostaining. For suspected glioma, genotyping for IDH1/2, TERT SNV, and CDKN2A copy number alteration was routinely performed, whereas H3F3A and BRAF SNV were assessed for selected cases. i-ID was determined based on the 2021 WHO classification and compared with the permanent integrated diagnosis (p-ID) to assess its reliability. RESULTS: After retrospectively analyzing 153 cases, 101 cases were prospectively examined using the i-ID system. Assessment of IDH1/2, TERT, H3F3AK27M, BRAFV600E, and CDKN2A alterations with i-ID and permanent genomic analysis was concordant in 100%, 100%, 100%, 100%, and 96.4%, respectively. Combination with FS and intraoperative genotyping assay improved diagnostic accuracy in gliomas. Overall, i-ID matched with p-ID in 80/82 (97.6%) patientswith glioma and 18/19 (94.7%) with PCNSL. CONCLUSIONS: The i-ID system provides reliable integrated diagnosis of adult malignant CNS tumors.

DOI： 10.1158/1078-0432.CCR-23-1660

PubMed

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本胆道学会  

researchmap

記述言語：英語  

Since the popularization of cancer screening and an improvement in treatment over the last two decades, multiple primary malignant neoplasms (MPMNs) have been increasingly reported. We report a patient who developed metachronous MPMNs in the breast, the endometrium, and the pancreas over a period of 13 years. A 42-year-old woman was first diagnosed with breast cancer and underwent breast-conserving surgery with adjuvant radiation therapy and endocrine therapy. Four years after breast surgery, she was diagnosed with endometrial cancer and underwent a laparoscopic modified radical hysterectomy with bilateral oophorectomy with pelvic lymph node dissection followed by adjuvant chemotherapy. However, there was peritoneal dissemination of endometrial cancer 1 year after surgery, which could be removed laparoscopically followed by adjuvant chemotherapy. Ten years after breast cancer surgery, pleural metastasis of breast cancer was diagnosed and treated by endocrine therapy. Thirteen years after breast cancer surgery, a pancreatic tumor with multiple liver masses emerged. It was difficult to diagnose whether primary or metastasis cancer by the results of the pathological analysis. Finally, we diagnosed primary pancreatic cancer with liver metastasis by clinical examination with the BRCA2-pathogenic variant. These tumors were well responded to chemotherapy and the patient survived during a follow-up period of 8 months. According to MPMNs, breast cancer patients should be followed-up carefully for the possibility of BRCA pathogenic variant and development of different primary malignant neoplasms.

DOI： 10.14740/wjon1658

PubMed

researchmap

記述言語：日本語   出版者・発行元：(公社)神奈川県医師会  

researchmap

記述言語：日本語   出版者・発行元：(公社)日本整形外科学会  

researchmap

記述言語：日本語   出版者・発行元：日本外科系連合学会  

researchmap

記述言語：日本語   出版者・発行元：日本脳腫瘍病理学会  

researchmap

記述言語：日本語   出版者・発行元：日本脳腫瘍病理学会  

researchmap

記述言語：日本語   出版者・発行元：日本脳腫瘍病理学会  

researchmap

記述言語：日本語   出版者・発行元：日本脳腫瘍病理学会  

researchmap

記述言語：日本語   出版者・発行元：(一財)日本消化器病学会  

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Biphenotypic sinonasal sarcoma is a newly established tumor entity that is associated with distinct clinicopathological findings. Biphenotypic sinonasal sarcoma is a rare, low-grade spindle cell sarcoma that arises in middle-aged females, exclusively in the sinonasal tract. A fusion gene involving PAX3 is detected in most biphenotypic sinonasal sarcomas, which aids in its diagnosis. Here, we report a case of biphenotypic sinonasal sarcoma with its cytological findings. The patient was a 73-year-old woman who presented with purulent nasal discharge and dull pain in the left cheek area. Computed tomography showed a mass extending from the left nasal cavity to the left ethmoid sinus, the left frontal sinus, and the frontal skull base. She underwent a combined transcranial and endoscopic approach for en bloc resection with a safety margin. Histologically, spindle-shaped tumor cells have been thought to proliferate mainly in the subepithelial stroma. Here, nasal mucosal epithelial hyperplasia was noted, and the tumor had invaded the bone tissue accompanying the epithelial cells. Fluorescence in situ hybridization (FISH) analysis showed a PAX3 rearrangement, and next-generation sequencing identified a PAX3::MAML3 fusion. Based on FISH, split signals were observed not in respiratory cells but in stromal cells. This indicated that respiratory cells were non-neoplastic. In the diagnosis of biphenotypic sinonasal sarcoma, the inverted growth of the respiratory epithelium can be a diagnostic pitfall. FISH analysis using a PAX3 break-apart probe is helpful not only for an accurate diagnosis but also for detecting the true neoplastic cells.

DOI： 10.1177/10668969231152577

PubMed

researchmap

記述言語：日本語   出版者・発行元：日本消化器病学会-関東支部  

researchmap

記述言語：日本語   出版者・発行元：医学図書出版(株)  

症例は61歳女性。左腎の水腎とサンゴ状結石を認め,腎瘻増設後に経皮・経尿道同時内視鏡手術(ECIRS)を施行された。その後も腎瘻からの粘液の排出が持続したため粘液産生腫瘍疑いで開腹左腎摘除術を施行した。病理診断は腎盂のvillous adenomaであった。術後2年6ヵ月後よりCTで残存尿管の拡張が認められた。腫瘍再発と考え開腹残存尿管摘除術を施行した。残存尿管には腸上皮化生が認められた。(著者抄録)

researchmap

記述言語：日本語   出版者・発行元：日本脳腫瘍病理学会  

researchmap

記述言語：日本語   出版者・発行元：医学図書出版(株)  

症例は76歳男性。CTで右水腎、腎盂尿管移行部の腫瘤を認め右腎盂癌cT3N0M0の診断となり、腹腔鏡下右腎尿管全摘術を施行した。組織所見ではIgG4陽性細胞を含む花筵状の膠原線維の増生を背景に過形成性のリンパ組織がみられ、形質細胞分化を伴ったB細胞性腫瘍を疑う細胞が認められた。IgG4関連疾患準確診およびIgG4関連腎臓病準確診例であり、それを背景としてindolent B細胞リンパ腫を生じたと考えられた。(著者抄録)

researchmap

記述言語：日本語   出版者・発行元：医学図書出版(株)  

症例は76歳男性。CTで右水腎、腎盂尿管移行部の腫瘤を認め右腎盂癌cT3N0M0の診断となり、腹腔鏡下右腎尿管全摘術を施行した。組織所見ではIgG4陽性細胞を含む花筵状の膠原線維の増生を背景に過形成性のリンパ組織がみられ、形質細胞分化を伴ったB細胞性腫瘍を疑う細胞が認められた。IgG4関連疾患準確診およびIgG4関連腎臓病準確診例であり、それを背景としてindolent B細胞リンパ腫を生じたと考えられた。(著者抄録)

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

PURPOSE: Molecular targeted therapy using BRAF and/or MEK inhibitors has been applied to BRAFV600E mutant high-grade gliomas (HGGs); however, the therapeutic effect is limited by the emergence of drug resistance. EXPERIMENTAL DESIGN: We established multiple paired BRAFV600E mutant HGG patient-derived xenograft (PDX) models based on tissues collected prior to and at relapse after molecular targeted therapy. Using these models, we dissected treatment resistant mechanisms for molecular targeted therapy and explored therapeutic targets to overcome resistance in BRAFV600E HGG models in vitro and in vivo. RESULTS: We found that, despite causing no major genetic and epigenetic changes, BRAF and/or MEK inhibitor treatment deregulated multiple negative feedback mechanisms, which led to the re-activation of the MAPK pathway through c-Raf and AKT signaling. This altered oncogenic signaling primarily mediated resistance to molecular targeted therapy in BRAFV600E mutant HGG. To overcome this resistance mechanism, we performed a high-throughput drug screening to identify therapeutic agents that potently induce additive cytotoxicity with BRAF and MEK inhibitors. We discovered that HSP90 inhibition combined with BRAF/MEK inhibition coordinately deactivated the MAPK and AKT/mTOR pathways, and subsequently induced apoptosis via dephosphorylation of GSK3β (Ser9) and inhibition of Bcl-2 family proteins. This mediated potent cytotoxicity in vitro and in vivo in refractory models with acquired resistance to molecular-targeted therapy. CONCLUSIONS: The combination of an HSP90 inhibitor with BRAF or MEK inhibitors can overcome the limitations of the current therapeutic strategies for BRAFV600E mutant HGG.

DOI： 10.1158/1078-0432.CCR-21-3622

PubMed

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：英語  

Serous endometrial intraepithelial carcinoma is the precursor of invasive uterine serous carcinoma. Here, we present two cases of serous endometrial intraepithelial carcinoma with omental micrometastasis and discuss their clinical significance. Two menopausal patients with abnormal endometrial biopsy findings underwent hysterectomy and comprehensive surgical staging (bilateral salpingo-oophorectomy, omentectomy, and pelvic and para-aortic lymphadenectomy). Although gross examination failed to detect tumors, the pathological diagnosis was serous endometrial intraepithelial carcinoma. Both patients had omental micrometastasis; they were diagnosed with International Federation of Gynecology and Obstetrics stage IVB disease and received postoperative chemotherapy. One patient died of the carcinoma 9 months after the hysterectomy, and the other had a recurrence of carcinoma 17 months after the end of the initial therapy. The present cases and literature review highlight the importance of meticulous inspection for micrometastasis in the abdominal cavity, including the omentum and peritoneum, for predicting prognosis.

DOI： 10.1111/jog.15020

PubMed

researchmap

記述言語：日本語   出版者・発行元：日本消化器病学会-関東支部  

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: The role of hepatic iron overload (HIO) in nonalcoholic fatty liver disease (NAFLD) pathogenesis has not been fully elucidated. PURPOSE: This study aimed to investigate the effect of HIO and examine the diagnostic usefulness of magnetic resonance imaging (MRI)-based R2* quantification in evaluating hepatic iron content (HIC) and pathological findings in NAFLD. STUDY TYPE: Prospective and retrospective. POPULATION: A prospective study of 168 patients (age, 57.2 ± 15.0; male/female, 80/88) and a retrospective validation study of 202 patients (age, 57.0 ± 14.4; male/female, 113/89) with liver-biopsy-confirmed NAFLD were performed. FIELD STRENGTH/SEQUENCE: 3 T; chemical-shift encoded multi-echo gradient echo. ASSESSMENT: Using liver tissues obtained by liver biopsy, HIC was prospectively evaluated in 168 patients by atomic absorption spectrometry. Diagnostic accuracies of HIC and R2* for grading hepatic inflammation plus ballooning (HIB) as an indicator of NAFLD activity were assessed. STATISTICAL TESTS: Student's t-test and analysis of variance (ANOVA) with Scheffe's multiple testing correction for univariate comparisons; multivariate logistic analysis. P-value less than 0.05 is statistically significant. RESULTS: HIC was significantly correlated with HIB grades (r = 0.407). R2* was significantly correlated with HIC (r = 0.557) and HIB grades (r = 0.569). R2* mapped an area under the receiver operating characteristic (AUROC; 0.774) for HIC ≥808 ng/mL (median value) with cutoff value of 62.5 s-1 . In addition, R2* mapped AUROC of HIB for grades ≥3 was 0.799 with cutoff value of 58.5 s-1 . When R2* was <62.5 s-1 , R2* correlated weakly with HIC (r = 0.372) as it was affected by fat deposition and did not correlate with HIB grades (P = 0.052). Conversely, when R2* was ≥62.5 s-1 , a significant correlation of R2* with HIC (r = 0.556) and with HIB grades was observed (P < 0.0001) with being less affected by fat deposition. DATA CONCLUSION: R2*  ≥ 62.5 s-1 is a promising modality for non-invasive diagnosis of clinically important high grades (≥3) of HIB associated with increased HIC. LEVEL OF EVIDENCE: 1 TECHNICAL EFFICACY STAGE: 2.

DOI： 10.1002/jmri.27810

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are fatal adverse skin reactions characterized by high fever, epidermal detachment, and mucositis. It is well known that SJS/TEN occasionally affects various organs, leading to permanent damage and death in some patients. Although acute liver dysfunction is a relatively common complication of SJS/TEN, severe acute liver dysfunction requiring liver transplantation is rare. We present the case of a 14-year-old girl with SJS complicated by severe and rapidly progressive liver dysfunction, specifically, acute liver failure (ALF) requiring liver transplantation. A lymphocyte transformation test showed positive results for acetaminophen and cefdinir. Furthermore, human leukocyte antigen (HLA) genotyping revealed the presence of the HLA-A*02:06 genotype, which is reported to be strongly associated with acetaminophen-related SJS/TEN with severe ocular complications. These results suggested that our patient may have presented with acetaminophen-induced SJS complicated by ALF, but no ocular complications. This is the first report of a pediatric patient with SJS who required liver transplantation. In rare instances, severe liver dysfunction requiring liver transplantation should be considered as a possible complication of SJS/TEN.

DOI： 10.1111/1346-8138.15963

PubMed

researchmap

記述言語：日本語   出版者・発行元：日本脳腫瘍病理学会  

researchmap

記述言語：日本語   出版者・発行元：日本脳腫瘍病理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本小児神経外科学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本小児神経外科学会  

researchmap

掲載種別：研究論文（学術雑誌）   出版者・発行元：BMJ  

<sec><title>Objectives</title>To determine clinical and genetic features of individuals with relapsing polychondritis (RP) likely caused by pathogenic somatic variants in ubiquitin-like modifier activating enzyme 1 (<italic>UBA1</italic>).

</sec><sec><title>Methods</title>Fourteen patients with RP who met the Damiani and Levine criteria were recruited (12 men, 2 women; median onset age (IQR) 72.1 years (67.1–78.0)). Sanger sequencing of <italic>UBA1</italic> was performed using genomic DNA from peripheral blood leukocytes or bone marrow tissue. Droplet digital PCR (ddPCR) and peptide nucleic acid (PNA)-clamping PCR were used to detect low-prevalence somatic variants. Clinical features of the patients were investigated retrospectively.

</sec><sec><title>Results</title><italic>UBA1</italic> was examined in 13 of the 14 patients; 73% (8/11) of the male patients had somatic <italic>UBA1</italic> variants (c.121A&gt;C, c.121A&gt;G or c.122T&gt;C resulting in p.Met41Leu, p.Met41Val or p.Met41Thr, respectively). All the variant-positive patients had systemic symptoms, including a significantly high prevalence of skin lesions. ddPCR detected low prevalence (0.14%) of somatic variant (c.121A&gt;C) in one female patient, which was subsequently confirmed by PNA-clamping PCR.

</sec><sec><title>Conclusions</title>Genetic screening for pathogenic <italic>UBA1</italic> variants should be considered in patients with RP, especially male patients with skin lesions. The somatic variant in <italic>UBA1</italic> in the female patient is the first to be reported.

</sec>

DOI： 10.1136/annrheumdis-2021-220089

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：ELSEVIER SCIENCE INC  

Vascular anomalies in infants and children were separated into hemangiomas and vascular malformations based on their clinical appearance, histopathologic features, and biologic behavior. Whereas vascular malformations are structural abnormalities of capillary, arterial, venous, or lymphatic elements, vascular tumors are characterized by increased endothelial cell turnover. However, misdiagnoses and misperceptions persist around the use of the term hemangioma: Although venous malformations arising in the submandibular gland are extremely rare, the most have been diagnosed as "hemangioma". We report a rare submandibular gland venous malformation with multiple phleboliths. A 54-year-old female visited a private dental clinic for the pain of the right mandibular third molar. Panoramic radiograph showed multiple radiopaque lesions below the right mandibular angle. After the diagnosis of submandibular gland stones, she was referred to our department for the treatment. Ultrasonography showed a heterogeneous, hypoechoic lesion with multiple hyperechoic lesions and blood flow in the right submandibular gland. CT showed a well-circumscribed, heterogenous, low-density lesion with multiple calcifications in the right submandibular gland. MRI showed a lesion with signal intensity similar to that of the muscle in the right submandibular gland had multiple low signal intensity lesions on gadolinium-enhanced T1-weighted images. Radiological diagnosis was submandibular gland venous malformation with multiple phleboliths. She underwent intraoral removal of submandibular gland lesion under general anesthesia. Pathological diagnosis was submandibular gland venous malformation with multiple phleboliths. (C) 2020 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd. All rights reserved.

DOI： 10.1016/j.ajoms.2020.10.006

Web of Science

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Partial deletions in chromosomes 1p and 19q are found in a subset of astrocytic tumors; however, it remains unclear how these alterations affect their histological features and prognosis. Herein, we present 3 cases of isocitrate dehydrogenase (IDH)-mutant astrocytoma with chromosome 19q13 deletion. In the first case, the primary tumor harbored an IDH1 mutation with chromosome 1p/19q partial deletions, which covered 19q13 and exhibited a durable initial response to radiotherapy and temozolomide (TMZ) treatment. However, the tumor lost the chromosome 1p/19q partial deletions at recurrence and became resistant to TMZ. Histologically, an oligodendroglioma-like feature was found in the primary tumor but not in the recurrent tumor. Capicua transcriptional repressor (CIC), located on 19q13, was less expressed in the primary tumor but was highly expressed in the recurrent tumor. Similar histological findings were observed in 2 other astrocytic tumors with IDH1 or IDH2 mutations. These tumors also had chromosome 19q13 deletion, including the CIC gene, weakly expressed CIC, and oligodendroglioma-like morphology. These tumors recurred at 6 and 32 months, respectively. These findings suggest that IDH-mutant astrocytoma with chromosome 19q13 partial deletion, including the CIC gene, may induce an oligodendroglioma-like phenotype, but the clinical prognosis may not be similar to that of genetically defined oligodendroglioma.

DOI： 10.1093/jnen/nlaa161

PubMed

researchmap

掲載種別：研究論文（学術雑誌）   出版者・発行元：Wiley  

DOI： 10.1002/ijc.33315

researchmap

その他リンク： https://onlinelibrary.wiley.com/doi/full-xml/10.1002/ijc.33315

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Actinomycotic osteomyelitis is an aggressive and persistent disease capable of invading and destroying bone, and chronic osteomyelitis with proliferative periostitis represents new bone formation with periosteal reaction. We report a rare case of actinomycotic osteomyelitis with proliferative periostitis arising in the mandibular ramus and spontaneous bone regeneration after coronoidectomy. A 14-year-old girl was referred for swelling in the right parotid-masseteric region and severe trismus. Contrast-enhanced CT revealed that heterogenous enhancement of the right masseter muscle, and a reactive bone formation over the lateral cortex of the right mandibular ramus and osteolysis of the condyle were seen in plain CT. MRI showed that the mandibular ramus was a low-signal intensity and the reactive bone on the ramus was signal intensity similar to muscle on T1-weighted images. The lesion was clinically and radiologically diagnosed as chronic osteomyelitis of the mandibular ramus. However, a biopsy was performed intraorally under general anesthesia to rule out a malignant bone tumor, and pathological examination showed fibrous bone and Actinomyces druses. Finally, the lesion was diagnosed as actinomycotic osteomyelitis with proliferative periostitis. She underwent image-guided intraoral removal of impacted right third molar and reactive proliferative bone on the right mandibular ramus under general anesthesia. To improve trismus, coronoidectomy also was performed. After the discharge, AMPC was administrated intraorally for 7.5 months. Postoperative panoramic radiograph and CT showed the right mandibular angle resorption and coronoid process regeneration. There was no recurrence of mandibular osteomyelitis 7 years after surgery.

DOI： 10.1007/s11282-020-00462-x

PubMed

researchmap

記述言語：英語  

Dermatofibrosarcoma protuberans (DFSP) originates from the dermal layer of the skin; the optimum treatment is an extended marginal resection. We describe a case of DFSP of the scalp with a skull invasive defect that was thoroughly examined pathologically to determine the optimum length of surgical margins. The tumor cells infiltrated up to 26 mm into the dermal tissues, whereas no infiltrating tumor cells were present in the skull, indicating the combination of marginal resection of the dermal tissues and lower of the skull can be a clinically relevant strategy for treatment of DFSP cases with skull invasion.

DOI： 10.2176/nmccrj.cr.2020-0297

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Primary central nervous system lymphoma (PCNSL) is an isolated type of lymphoma of the central nervous system and has a dismal prognosis despite intensive chemotherapy. Recent genomic analyses have identified highly recurrent mutations of MYD88 and CD79B in immunocompetent PCNSL, whereas LMP1 activation is commonly observed in Epstein-Barr virus (EBV)-positive PCNSL. However, a lack of clinically representative preclinical models has hampered our understanding of the pathogenic mechanisms by which genetic aberrations drive PCNSL disease phenotypes. Here, we establish a panel of 12 orthotopic, patient-derived xenograft (PDX) models from both immunocompetent and EBV-positive PCNSL and secondary CNSL biopsy specimens. PDXs faithfully retained their phenotypic, metabolic, and genetic features, with 100% concordance of MYD88 and CD79B mutations present in PCNSL in immunocompetent patients. These models revealed a convergent functional dependency upon a deregulated RelA/p65-hexokinase 2 signaling axis, codriven by either mutated MYD88/CD79B or LMP1 with Pin1 overactivation in immunocompetent PCNSL and EBV-positive PCNSL, respectively. Notably, distinct molecular alterations used by immunocompetent and EBV-positive PCNSL converged to deregulate RelA/p65 expression and to drive glycolysis, which is critical for intracerebral tumor progression and FDG-PET imaging characteristics. Genetic and pharmacologic inhibition of this key signaling axis potently suppressed PCNSL growth in vitro and in vivo. These patient-derived models offer a platform for predicting clinical chemotherapeutics efficacy and provide critical insights into PCNSL pathogenic mechanisms, accelerating therapeutic discovery for this aggressive disease. SIGNIFICANCE: A set of clinically relevant CNSL xenografts identifies a hyperactive RelA/p65-hexokinase 2 signaling axis as a driver of progression and potential therapeutic target for treatment and provides a foundational preclinical platform. GRAPHICAL ABSTRACT: http://cancerres.aacrjournals.org/content/canres/80/23/5330/F1.large.jpg.

DOI： 10.1158/0008-5472.CAN-20-2425

PubMed

researchmap

記述言語：日本語   掲載種別：研究論文（学術雑誌）  

A case of 69-year-old man underwent resection for the plantar surface of left foot malignant melanoma and received a sentinel biopsy of left inguinal lymph node. Two years and 10 months later, a mass of 30 mm in diameter in the ileum was detected by contrast-enhanced computed tomography, which showed abnormal uptake using FDG positron emission tomography. The partial intestinal resection was performed, and then, the mass was diagnosed as metastasis of malignant melanoma by pathological examination. Malignant melanoma is highly malignant disease that frequently shows distant metastasis. Although the malignant melanoma with distant metastasis shows poor prognosis, previous studies reported the prognosis could be improved when the patient could receive curative resection for single intraabdominal metastasis. Therefore, surgical resection should be considered for the single metastasis of malignant melanoma. We report a case of malignant melanoma with ileum metastasis resected curatively with literature review.

PubMed

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本小児神経外科学会  

researchmap

掲載種別：研究論文（学術雑誌）   出版者・発行元：Elsevier BV  

DOI： 10.1016/j.ajoms.2020.08.008

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

We present a case of a 14-year old boy with tumor-associated refractory epilepsy. Positron emission tomography imaging demonstrated a region with heterogeneous high 11C-methionine uptake and a region with homogenous low 18F-fluorodeoxyglucose uptake within the tumor. Histopathological and genomic analyses confirmed the tumor as BRAF V600E-mutated polymorphous low-grade neuroepithelial tumor of the young (PLNTY). Within the high-methionine-uptake region, we observed increased protein levels of L-type amino acid transporter 1 (LAT1), a major transporter of methionine; c-Myc; and constituents of the mitogen-activated protein kinase (MAPK) pathway. We also found that LAT1 expression was linked to the BRAF V600E mutation and subsequent activation of MAPK signaling and c-Myc. Pharmacological and genetic inhibition of the MAPK pathway suppressed c-Myc and LAT1 expression in BRAF V600E-mutated PLNTY and glioblastoma cells. The BRAF inhibitor dabrafenib moderately suppressed cell viability in PLNTY. Collectively, our results indicate that BRAF V600E mutation-activated MAPK signaling and downstream c-Myc induces specific metabolic alterations in PLNTY, and may represent an attractive target in the treatment of the disease.

DOI： 10.1186/s40478-020-01023-3

PubMed

researchmap

記述言語：日本語   出版者・発行元：日本脳腫瘍病理学会  

researchmap

記述言語：日本語   出版者・発行元：神奈川県医師会  

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：日本語   出版者・発行元：(公社)日本皮膚科学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：神奈川県医師会  

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Primary central nervous system lymphoma (PCNSL) is a rare and aggressive type of extranodal non-Hodgkin lymphoma that carries an unsatisfactory prognosis. Treating refractory PCNSL is challenging because of resistance to conventional cytotoxic and intrathecal chemotherapies. Therefore, novel therapeutic approaches are needed. Here, we report a 12-year-old boy with CD20-positive PCNSL, which was refractory to combination chemotherapy and intravenous rituximab. However, the patient achieved complete remission after repeated intraventricular rituximab administration. The results of this case indicate that intraventricular rituximab is an effective option to treat refractory PCNSL in children.

DOI： 10.1097/MPH.0000000000001291

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

PURPOSE: Oligodendroglioma has a relatively favorable prognosis, however, often undergoes malignant progression. We hypothesized that preclinical models of oligodendroglioma could facilitate identification of therapeutic targets in progressive oligodendroglioma. We established multiple oligodendroglioma xenografts to determine if the PI3K/AKT/mTOR signaling pathway drives tumor progression. EXPERIMENTAL DESIGN: Two anatomically distinct tumor samples from a patient who developed progressive anaplastic oligodendroglioma (AOD) were collected for orthotopic transplantation in mice. We additionally implanted 13 tumors to investigate the relationship between PI3K/AKT/mTOR pathway alterations and oligodendroglioma xenograft formation. Pharmacologic vulnerabilities were tested in newly developed AOD models in vitro and in vivo. RESULTS: A specimen from the tumor site that subsequently manifested rapid clinical progression contained a PIK3CA mutation E542K, and yielded propagating xenografts that retained the OD/AOD-defining genomic alterations (IDH1R132H and 1p/19q codeletion) and PIK3CAE542K, and displayed characteristic sensitivity to alkylating chemotherapeutic agents. In contrast, a xenograft did not engraft from the region that was clinically stable and had wild-type PIK3CA. In our panel of OD/AOD xenografts, the presence of activating mutations in the PI3K/AKT/mTOR pathway was consistently associated with xenograft establishment (6/6, 100%). OD/AOD that failed to generate xenografts did not have activating PI3K/AKT/mTOR alterations (0/9, P < 0.0001). Importantly, mutant PIK3CA oligodendroglioma xenografts were vulnerable to PI3K/AKT/mTOR pathway inhibitors in vitro and in vivo-evidence that mutant PIK3CA is a tumorigenic driver in oligodendroglioma. CONCLUSIONS: Activation of the PI3K/AKT/mTOR pathway is an oncogenic driver and is associated with xenograft formation in oligodendrogliomas. These findings have implications for therapeutic targeting of PI3K/AKT/mTOR pathway activation in progressive oligodendrogliomas.

DOI： 10.1158/1078-0432.CCR-18-4144

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND AND AIM: Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) presents as isolated proximal-type sclerosing cholangitis (i-SC). The present study sought to clarify the imaging differences between i-SC and Klatskin tumor. Differences between i-SC and IgG4-SC associated with autoimmune pancreatitis (AIP-SC) were also studied. METHODS: Differentiating factors between i-SC and Klatskin tumor were studied. Serum IgG4 level, CA19-9 level, computed tomography (CT) findings, cholangiography findings (symmetrical smooth long stricture extending into the upper bile duct [SSLS]), endosonographic features (continuous symmetrical mucosal lesion to the hilar part [CSML]), endoscopic biopsy results, treatment, relapse, and survival were also compared between patients with i-SC and those with AIP-SC. RESULTS: For a differential diagnosis between i-SC (N = 9) and Klatskin tumor (N = 47), the cut-off value of serum IgG4 level was 150 mg/dL (sensitivity, 0.857, specificity, 0.966). Logistic regression analysis indicated that serum IgG4 level, presence of SSLS, presence of CSML, and presence of swollen ampulla are independent factor for identifying i-SC. Relapse rate was significantly higher in the IgG4-SC with AIP group than in the i-SC group (log rank, P = 0.046). CONCLUSION: Isolated proximal-type sclerosing cholangitis presents as a nodular lesion with SSLS and/or CSML mimicking a Klatskin tumor. Those endoscopic features might provide a diagnostic clue for i-SC. i-SC is likely to have a more favorable prognosis than IgG4-SC with AIP.

DOI： 10.1111/den.13320

PubMed

researchmap

記述言語：日本語   出版者・発行元：(一社)関東連合産科婦人科学会  

researchmap

記述言語：英語  

INTRODUCTION: The World Health Organization (WHO) proposed an integrated classification for intraductal papillary neoplasm of the bile duct (IPNB) in 2010. However, IPNB reportedly shows considerable geographic variation. This Japanese single-institution study examined outcomes of surgery for IPNB and the prognostic impact of immunohistochemical mucin expression patterns. MATERIALS AND METHODS: Patients with IPNB were identified from 413 patients who underwent curative-intent surgery for biliary tract (excluding gallbladder) neoplasms from 1992 to 2016 by retrospective macro- and microscopic reevaluation of resected specimens. Their clinicopathological variables were analyzed. RESULTS: Twenty-two (5%) 2010 WHO classification-based patients with IPNB were identified. The other 391 patients had common-type cholangiocarcinoma. The histopathological grade was low/intermediate in 2 patients (9%), high in 8 (36%), and invasive carcinoma (ICa) in 12 (55%). The 10-year overall survival rate was 100% in 10 patients with low-high grade IPNB and 69% in 12 patients with ICa. These rates were significantly (p = 0.018) or marginally (p = 0.089) better than that (38%) of 391 other-cholangiocarcinoma patients. In the 12 patients with ICa, R0 or R1 resection, MUC5AC, and MUC6 expression significantly affected survival. Notably, all seven patients with ICa exhibiting MUC5AC expression survived throughout the study period, while four of five patients with ICa who did not exhibit MUC5AC expression died of recurrence (with vs. without MUC5AC: 10-year overall survival, 100% vs. 60%, respectively; p = 0.018). CONCLUSION: Our 24-year, single institution's experience suggests that Japanese patients with IPNB favorably respond to surgery, even with ICa. MUC5AC and MUC6 expression may be predictive of favorable outcomes.

DOI： 10.1016/j.ejso.2018.10.532

PubMed

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本外科学会  

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Background: Application of next-generation DNA sequencing (NGS) has recently become increasingly common in the field of clinical oncology in several countries around the world. In Japan also, a system for applying NGS to routine clinical practice is gradually being established. During this process, we introduced in Japan the tumor-profiling MSK-IMPACT (Memorial Sloan Kettering-Integrated Mutation Profiling of Actionable Cancer Targets) assay. Methods: We present here our initial experience with the use of MSK-IMPACT in 68 patients selected from two institutions in Japan between June 2016 and October 2017. Results: MSK-IMPACT sequencing was successful and yielded results in specimens obtained from 64 of the 68 patients, representing an overall assay success rate of 94.1%. The top three cancer types tested were endometrial cancer (17.2%), pancreatic cancer (15.6%) and colorectal cancer (12.5%). Evaluation of the clinical actionability of the genetic alterations revealed that 25.0% of patients (n = 16) harbored at least one actionable alteration. However, enrolling the patients in a genomically matched clinical trial was difficult, mainly because most clinical trials are limited to tumors arising from a specific organ/site. One patient with microsatellite instability-high status, as determined by MSK-IMPACT, was treated with pembrolizumab and showed partial response. Conclusions: Although tumor profiling by NGS and administration of genomically matched therapy is a promising strategy, because of its high cost, we need to consider how we can fit it into the Japanese medical system. Towards this end, we believe that it is important to share our initial experience for furthering precision medicine in Japan.

DOI： 10.1093/jjco/hyy173

PubMed

researchmap

記述言語：英語  

In the revised World Health Organization classification 2016, anaplastic pleomorphic xanthoastrocytoma (PXA) has been newly defined as a variant of the PXA entity. Furthermore, some anaplastic PXA were reported to have extremely poor prognosis which showed a type of pediatric glioblastoma (GBM) molecular profile. Recent integrated molecular classification for primary central nervous system tumors proposed some differences between histological and molecular features. Herein, in a genome-wide molecular analysis, we show an extreme aggressive anaplastic PXA that resulted in a pediatric GBM molecular profile. A full implementation of the molecular approach is the key to predict prognosis and decide the treatment strategy for anaplastic PXA.

DOI： 10.1111/cas.13903

PubMed

researchmap

記述言語：日本語   出版者・発行元：神奈川県医師会  

researchmap

記述言語：日本語   出版者・発行元：神奈川県医師会  

researchmap

記述言語：日本語   出版者・発行元：神奈川県医師会  

researchmap

記述言語：日本語   出版者・発行元：神奈川県医師会  

researchmap

記述言語：日本語   出版者・発行元：日本外科系連合学会  

症例は54歳女性．既往として神経線維腫症Ⅰ型：von Recklinghausen病（以下vRD）と診断されていた．血便を主訴に他院を受診し，上下部内視鏡が施行されたが異常なく，当院紹介となった．カプセル内視鏡検査で，上部小腸に小腸腫瘍を疑わせる所見を認めた．経口および経肛門小腸鏡が施行され，gastrointestinal stromal tumor（以下GIST）と診断された．腹腔鏡補助下での小腸部分切除術および腫瘍切除術を施行した．腫瘍は空腸から回腸までに存在し，２mmから50mmまでで，計12個を確認した．いずれもが小腸GISTであった．vRDには近年GIST合併例の報告が散見される．今回われわれは稀なvRDに合併した多発生小腸GISTを腹腔鏡補助下に切除を行った１例を経験した．この症例のような多発する特徴のある病変に対し，腹腔鏡による観察は有用であると考え報告する．

DOI： 10.4030/jjcs.44.49

CiNii Research

researchmap

その他リンク： https://search.jamas.or.jp/link/ui/2019130431

掲載種別：研究論文（学術雑誌）   出版者・発行元：Medknow  

DOI： 10.4103/ajns.ajns_158_19

researchmap

記述言語：日本語   出版者・発行元：(一社)日本消化器外科学会  

researchmap

記述言語：日本語   出版者・発行元：(公社)日本皮膚科学会  

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：英語   出版者・発行元：(一社)日本癌治療学会  

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Sandhoff disease (SD) is a genetic disorder caused by a mutation of the β-subunit gene β-hexosaminidase B (HexB) in humans, which results in the massive accumulation of the ganglioside GM2 and related glycosphingolipids in the nervous system. SD causes progressive neurodegeneration and changes in white matter in human infants. An animal model of SD has been established, Hexb-deficient (Hexb) mice, which shows abnormalities resembling the severe phenotype found in human infants. Previously, we reported that the activation state of microglia caused astrogliosis in the early stage of Hexb mouse development. To study how the symptoms of SD develop, we explored the difference in gene expression between 4-week-old Hexb and Hexb mouse cerebral cortices by microarray analysis. The data indicated not only the upregulation of immune system-related genes but also the downregulation of myelin-related genes in the 4-week-old Hexb mouse cerebral cortices. To test the correlation between inflammation and dysmyelination, we generated double-knockout mice of Hexb and the Fc receptor γ gene (Fcrγ), which is a regulator of autoimmune responses. Dysmyelination recovered in these double-knockout mice. The number of oligodendrocyte progenitors, which expressed platelet-derived growth factor receptor-α, did not change in the 2-week-old mouse brain. These results indicate that microglial activation plays an important role in the myelination process, without influencing the number of oligodendrocyte progenitors, in the development of Hexb mice.

DOI： 10.1097/WNR.0000000000001060

PubMed

researchmap

記述言語：日本語   出版者・発行元：神奈川県医師会  

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

OBJECTIVE: The purpose of this study is to validate the applicability of new TNM classification for human papillomavirus (HPV)-related oropharyngeal cancer (OPC) in the 8th edition of the American Joint Committee on Cancer (AJCC)/Union for International Cancer Control (UICC) TNM staging system in Japan. METHODS: A total of 91 OPC patients treated with radiation-based therapy between November 2001 and July 2015 were analyzed retrospectively in this study. HPV infection status was evaluated using tumor p16 expression. RESULTS: 40 OPC patients (44.0%) had HPV-positive disease in this study. The distribution of disease stage of HPV-positive OPC patients dramatically changed from the 7th edition to the 8th edition of AJCC/UICC TNM classification. However, neither the 8th edition nor the 7th edition of the AJCC/UICC TNM staging system could adequately predict outcomes of HPV-positive OPC patients in our patient series. On the other hand, our multivariate analysis indicated that matted nodes and age ≥63 were independent prognostic factors for progression-free survival. In addition, HPV-positive OPC patients with stage I without matted nodes showed significantly better overall and progression-free survival compared with those with stage I with matted nodes and stages II and III in the 8th edition of the AJCC/UICC TNM staging system (P=0.008, and P=0.043, respectively). CONCLUSION: Our results suggested that matted nodes of HPV-positive OPC patients might be additionally examined to apply the 8th edition of AJCC/UICC TNM classification for more adequate predicting outcomes of HPV-positive OPC patients.

DOI： 10.1016/j.anl.2017.07.010

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Ciliated muconodular papillary tumors (CMPTs) are a recently categorized benign or low-grade malignant neoplasm that develops in the peripheral lung. Only about 40 cases have been reported to date, and the clinicopathological characteristics have yet to be defined in detail. Here, we present four cases of CMPTs with a focus on their immunohistochemical profiles and driver gene mutations. These tumors were a papillary proliferation of a mixture of ciliated, mucous, and basal cells located in the peripheral lung. Ciliated, mucous and basal cells were positive for TTF-1 when using the clone SPT24, but negative for HNF-4α. Basal cells were positive for p40. Mucous cells in some tumors were positive for MUC5AC and MUC6. The Ki-67 index was less than 5%, and strong expression of p53 was not detected. Three of the four tumors had a BRAF (V600E) driver mutation, an EGFR (del E746-T751/S752V) driver mutation, or driver mutations in both EGFR (E709G) and KRAS (G12V). These mutation types are rare for any histological type of lung cancer. The present results confirmed that CMPT is a neoplasm with immunohistochemical features and driver gene mutations that are distinct from those of common lung tumors.

DOI： 10.1111/pin.12664

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Paragangliomas are generally benign, slow-growing tumors. However, approximately 10%-20% are malignant, characterized by distant metastasis. Recently, a germ line mutation in succinate dehydrogenase B subunit (SDHB) has been shown to be associated with malignant behavior in paraganglioma. Here we present a case of SDHB-negative malignant paraganglioma of the jugular foramen with a pseudohypoxic microenvironment and unique imaging features on [18F]-fluoro-2-deoxy-d-glucose positron emission tomography ([18F]-FDG PET), and discuss the significance of SDHB immunohistochemistry and the potential of [18F]-FDG PET for clinical management. CASE DESCRIPTION: A 55-year-old woman was diagnosed with jugular foramen paraganglioma. Initial surgical resection was performed; however, follow-up [18F]-FDG PET indicated multiple uptake regions throughout the body. Biopsies for multiple recurrent lesions revealed consistent pathological features, suggesting distant metastasis. Immunohistochemical analysis revealed a lack of SDHB immunostaining in all specimens. Pseudohypoxic markers, including hypoxia-inducible factor-1α and downstream glycolysis enzymes, were strongly expressed. [18F]-FDG PET demonstrated increased uptake in the lesions, and the patient died 3 years after initial metastasis. CONCLUSION: In patients with head and neck paraganglioma without SDHB expression, close follow-up should be considered because of the risk for metastasis. In such cases, [18F]-FDG PET might be useful for detecting metastasis due to atypical accumulation from pseudohypoxia-induced glycolysis.

DOI： 10.1016/j.wneu.2018.02.147

PubMed

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：Elsevier Inc  

Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare complication of cancer. It is histologically characterized by fibrocellular intimal proliferation in small pulmonary arteries and arterioles in patients with metastatic carcinoma. It is most frequently caused by adenocarcinoma. We report a case of PTTM caused by uterine carcinosarcoma in a 62-year-old female, who exhibited reductions in blood pressure and oxygen saturation. This is the first case report about uterine carcinosarcoma-induced PTTM. Histologically, the primary uterine carcinosarcoma included serous carcinoma and homologous sarcoma components. Many tumor emboli were observed across large regions of both lungs. PTTM was found in the lower lobes. Interestingly, the embolized tumor cells were all derived from the adenocarcinoma component. Immunohistologically, the primary carcinosarcomaexhibited weak positivity for tissue factor (TF), but was negativefor vascular endothelial growth factor-A (VEGF-A). The PTTM tumor cells were positive for TF and weakly positive for VEGF-A. This case suggests that any tumor containing adenocarcinoma can cause PTTM, and immunostaining of the primary tumor might be worthless for determining whether PTTM will develop. PTTM should always be considered during the differential diagnosis of dyspnea in patients with adenocarcinoma.

DOI： 10.1016/j.ehpc.2017.11.003

Scopus

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

AIMS: Psammoma bodies are concentrically lamellated microscopic structures made of calcium. They are commonly observed in papillary carcinomas of the thyroid gland and serous papillary adenocarcinomas of the ovary, but are also occasionally detected in lung adenocarcinomas. Only one study, published in 1972, has systematically described the significance of psammoma bodies in lung adenocarcinomas. The aim of this study was to update the significance of psammoma bodies in lung adenocarcinomas from a modern perspective. METHODS AND RESULTS: Psammoma bodies were detected in 7.2% (59/822) of the adenocarcinomas examined, among which the papillary (20.3%, 12/59) and acinar (44.1%, 26/59) histological subtypes, with the feature of a terminal respiratory unit (91.5%, 54/59), were dominant. Malignant potential (cell growth activity measured by Ki67 labelling, lymph node metastasis, and postoperative survival) did not significantly differ between adenocarcinomas with and without psammoma bodies. On the basis of cytogenetic features, adenocarcinomas with psammoma bodies were preferentially affected by tyrosine kinase inhibitor (TKI)-targetable driver mutations [EGFR (69.8%, 37/53), ALK (13.2%, 7/53), and ROS1 (1.9%, 1/53)]. Multivariate analyses confirmed that psammoma bodies may constitute an independent predictor for these mutations, particularly EGFR and ALK mutations. CONCLUSIONS: Psammoma bodies may predict a favourable response of lung adenocarcinomas to TKIs.

DOI： 10.1111/his.13397

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Mutations in the MATR3 gene have been identified as a cause of familial amyotrophic lateral sclerosis, but involvement of the matrin 3 (MATR3) protein in sporadic amyotrophic lateral sclerosis (SALS) pathology has not been fully assessed. We immunohistochemically analyzed MATR3 pathology in the spinal cords of SALS and control autopsy specimens. MATR3 immunostaining of the motor neuron nuclei revealed two distinct patterns: mild and strong staining. There were no differences in the ratio of mild versus strong nuclear staining between the SALS and control cases. MATR3-containing neuronal cytoplasmic inclusions (NCIs) were observed in 60% of SALS cases. Most motor neurons with MATR3-positive NCIs exhibited a mild nuclear staining pattern. Although 16.8% of NCIs positive for transactivating response region DNA-binding protein 43 (TDP-43) were estimated as double-labeled by MATR3, no MATR3-positive or TDP-43-negative NCIs were observed. Although a previous study found that MATR3-positive NCIs are present only in cases with C9orf72 hexanucleotide repeat expansion, ubiquitin-positive granular NCIs were not observed in the cerebellum, which have been reported as specific to C9orf72-related ALS. Six ALS cases were confirmed to be negative for the GGGGCC hexanucleotide. Our results reveal that MATR3 is a component of TDP-43-positive NCIs in motor neurons, even in SALS, and indicate the broader involvement of MATR3 in ALS pathology and the heterogeneity of TDP-43-positive NCIs.

DOI： 10.1016/j.ajpath.2017.10.007

PubMed

researchmap

記述言語：日本語   掲載種別：研究論文（学術雑誌）  

The standard therapy for Stage IV gastric cancer is chemotherapy. It is not certain, but conversion surgery is expected to be effective. We report the cases of 3 patients who achieved long-term survival after conversion surgery. Case 1 was of a 59- year-old woman. The tumor was classified as L-Less Post, Type 2, tub1, cT3N2M1(#16a2lat), Stage IV. Then, we initiated S-1 plus cisplatin and the LN achieved PRafter 4 courses. We performed distal gastrectomy with D2 lymph node dissection in February 2011. It was classified as ypT2N2 and the primary lesion was histologically classified as Grade 1a. Case 2 was of a 74- year-old man. The tumor was classified as UM-Less Ant, Type 3, por1, cT3N2H0P1CY1, Stage IV. Then, we initiated docetaxel plus cisplatin plus S-1 and the primary tumor achieved PRafter 6 courses. There were no new tumors and we conducted a laparoscopic examination. After the decision of P0CY0, we performed total gastrectomy with D2 lymph node dissection in April 2012. It was classified as ypT3N1 and the primary lesion was histologically classified as Grade 2. Case 3 was of a 64-yearold woman. The tumor was classified as UM-Less, Type 3, por1, cT3N2H1M0(liver), Stage IV. Then, we initiated capecitabin plus cisplatin and liver metastasis achieved PRafter 6 courses. We performed total gastrectomy with D2 lymph node dissection in July 2012. It was classified as ypT3N1 and the primary lesion was histologically classified as Grade 1b. All postoperative chemotherapy courses were of only S-1. In case 1, the para aortic LN exhibited recurrence 6 months postoperatively. We initiated weekly paclitaxel as second-line therapy. It achieved CRafter 6 courses, and the same trend was maintained. In cases 2 and 3, no therapy was administered after 8 S-1 courses, but no recurrences occurred. All patients survived after 62-77 months postoperatively. A new clinical trial is needed to prove the improvement in prognosis for Stage IV gastric cancer after conversion surgery.

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Stomach cancer rarely develops in patients with familial adenomatous polyposis (FAP), and Helicobacter pylori infection may increase the risk of FAP-related gastric cancer. We describe the case of a 64-year-old woman who developed multiple synchronous early gastric cancers without H. pylori infection. Nine cancer lesions were successfully treated by endoscopic submucosal dissection. An immunohistochemical analysis revealed that the tumors were positive for mucin (MUC)2, MUC6, and CDX2, but negative for MUC5AC, suggesting that the tumors were gastrointestinal mixed type. Periodical endoscopic surveillance is important for the detection of cancers at an early stage.

DOI： 10.2169/internalmedicine.8735-16

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：Elsevier Ltd  

We determined high concentrations of arginine and polyamines (putrescine, spermidine, and spermine) in yuzu (Citrus junos) seeds. Yuzu produces large quantities of limonoids. We fed limonoid aglycones extracted from yuzu seeds and commercial spermine as a polyamine to Sandhoff disease (SD) mice with abnormal glycolipid metabolism and autosomal recessive inheritance, as found in humans. Ingestion of limonoid aglycones and spermine by SD mice extended their longevity by about 10–12%, where it alleviated inflammation of the central nervous system. After administering limonoid aglycones or limonoid aglycones/spermine, analyses of the bacterial flora in the feces based on 16S rDNA indicated changes in the relative ratio between Bacteroidetes (Bacteroidales and Lactobacillus) and Firmicutes (Clostridiales and Erysipelotrichaceae). In both SD and wild-type mice, the amount of short-chain fatty acids produced by the bacterial flora increased according to the intake of limonoid aglycones and spermine.

DOI： 10.1016/j.jff.2017.09.043

Scopus

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Succinate dehydrogenase-deficient renal cell carcinoma (SDH-deficient RCC) is a newly introduced histological type of RCC, which is caused by loss of subunit genes of SDH. It is known to frequently demonstrate familial occurrence and be frequently associated with gastrointestinal stromal tumors and paraganglioma. To date, only 53 cases have been reported. Here, we present an additional case of SDH-deficient RCC occurring in a 40-year-old female. The tumor was histologically biphasic, consisting of tubular and solid architectures. The tumor cells possessed oval nuclei with small nucleoli, and an eosinophilic granular cytoplasm with occasional vacuoles. These cells completely lost the immunohistochemical expression of B subunit of SDH (SDHB). Consequently, the tumor was diagnosed as SDHB-deficient RCC. We identified a novel germ line mutation of the SDHB gene, and also confirmed a hemizygous deletion of the wild-type allele in the tumor cells. To define the pathological characteristics of SDH-deficient RCC, precise diagnosis and accumulation of more cases are required.

DOI： 10.1111/pin.12587

PubMed

researchmap

記述言語：日本語   出版者・発行元：(株)癌と化学療法社  

症例は65歳、女性。胃癌(L.、less、type2、tub1、cT3、cN0、cH0、cP0、cM0、CY0、cStage IIA)の診断で、胃切除の治療方針となった。手術は幽門側胃切除術(D2郭清)およびBillroth I法再建を施行した。病理結果はML、less、type2、tub1、pT3、ly:1、v:1、N0、H0、P0、M0、CY0、pStage IIAであった。術後補助療法は施行せず、外来経過観察となった。術後5年目の腹部造影CT検査で臍部での再発を疑われ、生検の結果、胃癌臍転移の診断となった。再発後、S-1(80mg/m2、day 1〜21)/CDDP(60mg/m2、day 8、3週投与2週休薬)を7コース投与したが、有害事象(下痢:Garde 3)のため中止となった。その後、paclitaxel(80mg/m2、day 1、8、15:3週投与1週休薬)を33コース施行した。臍転移は画像上CRとなり、他臓器への転移もみられなかった。再発後3年以上経過した現在も外来通院中である。今回、化学療法が奏効し長期生存が得られた胃癌術後臍転移の1例を経験したので、若干の文献的考察を加えて報告する。(著者抄録)

researchmap

その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2017&ichushi_jid=J00296&link_issn=&doc_id=20171221480115&doc_link_id=%2Fab8gtkrc%2F2017%2F004412%2F115%2F1393-1395%26dl%3D0&url=http%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fab8gtkrc%2F2017%2F004412%2F115%2F1393-1395%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

記述言語：英語  

BACKGROUND: Primary large cell neuroendocrine carcinoma of the kidney is a rare and generally very aggressive disease. We present a case of a patient with primary large cell neuroendocrine carcinoma of the kidney with cardiac metastasis. CASE PRESENTATION: A 59-year-old Japanese man presented to his previous physician with hematuria. Computed tomography revealed masses in the heart and right kidney, and fluorodeoxyglucose-positron emission tomography showed abnormal uptake in the heart. A cardiac biopsy under transesophageal echocardiographic guidance revealed a metastatic tumor. Subsequently, multiple lung lesions were detected, and a right nephrectomy was performed after these metastases were suspected to have originated from renal carcinoma. Large cell neuroendocrine carcinoma of the kidney was ultimately diagnosed. Pancreatic metastasis was detected on computed tomography postoperatively. Three courses of chemotherapy with carboplatin and irinotecan were administered, and were temporarily effective against the metastatic lesions in the lungs and pancreas. However, our patient's general condition deteriorated with the progression of the lesions, and he died 9 months after his initial examination. CONCLUSIONS: Multi-agent chemotherapy, including platinum-based drugs was effective against large cell neuroendocrine carcinoma metastases, albeit only temporarily. This is the first reported case of large cell neuroendocrine carcinoma with cardiac metastasis.

DOI： 10.1186/s13256-017-1460-7

PubMed

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Ependymomas are primary neuroepithelial malignancies that mainly occur during childhood, and arise from ependymal cells along the ventricular systems of the CNS. Recently, it was elucidated that two-thirds of supratentorial (ST) ependymomas harbor oncogenic fusions of RELA, whose protein product is the principal effector of canonical NF-κB signaling. RELA fusion proteins activate signaling for tumor proliferation and malignant progression, resulting in poorer prognoses in these patients compared to those in patients with other ST ependymomas. In this study, we encountered a case of C11orf-RelA fusion-positive ST anaplastic ependymoma that was diagnosed in first tumor resection surgery of multi-staged gross total resection with molecular evidence. In ependymomas, regardless of tumor location or pathological grade, subtotal resection is associated with higher rates of mortality compared with GTR. Molecular analysis based on the application of recent molecular knowledge for ST ependymomas performs a role in appropriate and individualized treatment strategies.

DOI： 10.1007/s10014-017-0297-5

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Intracranial mycotic aneurysm (IMA) is a rare neurovascular disease and a well-known complication after infective endocarditis. IMAs potentially carry a high mortality risk resulting from intracranial hemorrhage. Therefore, initial treatment is crucial for IMA patients, but an optimal treatment strategy remains unknown. Herein, we report 1 cases of IMA patients treated with the current usual modalities, and we provide a comprehensive literature review to propose an optimal initial treatment strategy for IMAs. CASE DESCRIPTIONS: Case 1: An 80-year-old man received a diagnosis of ruptured IMA. He immediately underwent trapping surgery and was discharged without neurologic deficit. Case 2: A 36-year-old man with previous aortic root replacement received a diagnosis of ruptured IMA. His general condition was considered too unstable to allow him to undergo direct surgery, and the angiographic access route was limited because of the previous aortic replacement surgery. Therefore, we selected conservative therapy; however, the patient subsequently died after complications from a huge intracerebral hemorrhage during medical treatment. CONCLUSIONS: On the basis of 129 IMA cases across 54 reports published from 2006 to 2016, we propose initial surgical intervention as an optimal treatment for patients with ruptured, and even unruptured, IMAs. Regarding surgical intervention, there was no significant difference in postoperative modified Rankin scale scores between direct surgery and endovascular treatment. By contrast, because antibiotic treatment significantly decreased IMA size in unruptured IMAs, antibiotic treatment might be a reasonable alternative for patients with unruptured IMAs, depending on the patient's situation.

DOI： 10.1016/j.wneu.2017.07.016

PubMed

researchmap

記述言語：英語   出版者・発行元：日本癌学会  

researchmap

記述言語：英語   出版者・発行元：日本癌学会  

researchmap

記述言語：日本語   出版者・発行元：(公社)日本皮膚科学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

症例はいずれも50歳代女性。左肺底動脈大動脈起始症のため、左肺下葉切除が行われた。胸部下行大動脈から分岐した異常血管が肺底区に分布し、肺底区の肺動脈は欠損していた。異常血管は弾性動脈様の形態をとり、蛇行部で粥状硬化がみられた。末梢では肺高血圧症でみられる異常に類似した血管内腔の狭小化や閉塞、血管周囲の線維化を伴っていた。叢状病変は一方のみでみとめた。肺静脈は拡張とうっ血が目立ち、狭窄・閉塞はなかった。胸膜直下を中心に、胸膜に分布する体循環由来の血管が閉塞したと思われる結節状平滑筋増生がみられた。(著者抄録)

researchmap

記述言語：日本語   出版者・発行元：(一社)日本消化器外科学会  

症例は60歳の男性で,早期胃癌と診断し腹腔鏡補助下幽門保存胃切除術を施行(D1郭清)した.術後6年5ヵ月のCTで総肝動脈周囲(No.8a)リンパ節に孤立性の腫大を認め胃癌再発が疑われた.遠隔転移を認めず開腹No.8aリンパ節郭清を施行した.病理組織学的検査でカルチノイドと診断,PETや上下部消化管・小腸内視鏡検査を行ったが原発巣は不明であった.半年毎に画像検索し,リンパ節郭清後2年6ヵ月の上部消化管内視鏡検査で十二指腸球部に粘膜下腫瘍を認め生検でカルチノイドと診断した.リンパ節郭清後2年7ヵ月で開腹幽門側胃切除・十二指腸球部切除術(No.5,12a,17リンパ節郭清)を施行した.病理組織学的検査でリンパ節転移は認めなかった.最大腫瘍径20mm,核分裂像1個/HPF(high power field),MIB-1 index 2%でneuroendocrine tumor(NET)G1と診断した.リンパ節転移切除後30ヵ月で原発巣が判明した症例を経験したので報告する.(著者抄録)

researchmap

その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2017&ichushi_jid=J01117&link_issn=&doc_id=20170725270003&doc_link_id=10.5833%2Fjjgs.2014.0114&url=https%3A%2F%2Fdoi.org%2F10.5833%2Fjjgs.2014.0114&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_3.gif

記述言語：日本語   出版者・発行元：(一社)日本リンパ網内系学会  

researchmap

記述言語：英語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：英語   出版者・発行元：SPRINGER  

DOI： 10.1007/s00401-016-1664-8

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1038/srep40518

Web of Science

PubMed

researchmap

記述言語：日本語   掲載種別：研究論文（学術雑誌）   出版者・発行元：Japan Society of Hepatology  

We report a case of primary hepatic neuroendocrine carcinoma in a 77-years-old male. He presented with right hypochondriac pain. The serum was negative for hepatitis B surface antigen, and for hepatitis C virus antibody. US demonstrated low echoic nodule. Plain CT showed a low density. MRI showed hyperintensity on diffusion weighted imaging. Tumor biopsy was performed for definitive diagnosis. The tumor was diagnosed pathologically as a small cell carcinoma. He presented with flushing and intermittent bronchial wheezing in the hospital course. The symptoms were diagnosed as carcinoid syndrome by elevated 5-HIAA levels in the urine. Primary hepatic neuroendocrine carcinoma is rare, and so we report this case which appears to be of primary hepatic neuroendocrine carcinoma with carcinoid syndrome.

DOI： 10.2957/kanzo.58.632

Scopus

researchmap

記述言語：日本語   掲載種別：研究論文（学術雑誌）   出版者・発行元：一般社団法人 日本泌尿器科学会  

粘液管状紡錘細胞癌は2004年にWHO分類に加えられた腎癌組織型である．我々はMTSCCの有転移症例で，テムシロリムスが一時的だが奏効した症例を経験したので報告する．症例は45歳の女性．呼吸困難，体重減少，食思不振を主訴に当院を紹介受診した．造影CTで遅延型造影パターンを示す右腎腫瘍を認めた．腫瘍は下大静脈と左腎静脈内に腫瘍塞栓を形成し左腎に直接浸潤しており，肝臓と胃に転移を認めた．経皮的腎生検で，病理組織学的に粘液管状紡錘細胞癌（mucinous tubular and spindle cell carcinoma）と診断された．全身状態不良かつ非淡明細胞型腎細胞癌であったことからテムシロリムスによる治療を開始した．治療開始5週目に肝転移は著明に縮小し，食思不振は改善した．7週目には腫瘍塞栓の増大によりProgression Diseaseとなったためアキシチニブに治療を変更するも，診断後約6カ月で癌死した．粘液管状紡錘細胞癌は比較的予後良好と考えられていたが，本症例のように急速に悪化する症例も存在し必ずしも低悪性度ではないことが知られてきた．本症例でテムシロリムスが粘液管状紡錘細胞癌に対する治療の選択肢となり得ることが示唆された.

DOI： 10.5980/jpnjurol.108.149

PubMed

researchmap

記述言語：日本語   掲載種別：研究論文（学術雑誌）   出版者・発行元：Japanese Society of Gastroenterological Surgery  

A 60-year-old man was treated with laparoscopy-assisted pylorus-preserving gastrectomy for early gastric cancer. After surgery, the lesion was diagnosed as T1a N0M0, Stage IA. Six years and five months after the operation, we detected lymph node swelling around the common hepatic artery (No. 8a) and diagnosed lymph node metastasis. We performed No. 8a lymph node dissection, on the grounds of the lack of distant metastasis and the isolated lymph node swelling. The pathological findings showed a neuroendocrine tumor (NET). Therefore, we performed wholebody PET, gastrointestinal fiberscopy (GIF), colonofiberscopy (CF) and enteroscopy
however, no primary lesion was identified. We continued to evaluate the patient using imaging performed every six months. A total of 30 months after the No. 8a lymph node dissection, we diagnosed NET of the duodenum based on an endoscopic biopsy and performed distal gastrectomy and duodenal bulb resection with No. 5, 12a and 17 lymph node dissection. A histopathological inspection showed no metastasis of the lymph nodes. We herein report an interesting case in which the site of the primary lesion became clear approximately 30 months after the dissection of isolated lymph node metastasis.

DOI： 10.5833/jjgs.2014.0114

Scopus

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1159/000470838

PubMed

researchmap

記述言語：日本語   出版者・発行元：(一社)日本血液学会-東京事務局  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本癌治療学会  

researchmap

記述言語：英語   出版者・発行元：日本癌学会  

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1016/j.expneurol.2016.07.011

Web of Science

PubMed

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：CIG MEDIA GROUP, LP  

We here, for the first time, report that the risk of accidental tumor incision (ATI) during laparoscopic partial nephrectomy is influenced by the presence of a pseudocapsule, and, to some degree, by the tumor size. Moreover, we also show that ATI during laparoscopic partial nephrectomy is not necessarily associated with poor outcomes such as local tumor recurrence.
Background: To investigate the impact of accidental tumor incision (ATI) during laparoscopic partial nephrectomy (LPN) on the treatment outcome of LPN and to determine the predictive factors for ATI. Patients and Methods: Consecutive 156 patients with renal tumors suspicious of renal cell carcinoma cT1N0M0 undergoing laparoscopic partial nephrectomy at Yokohama City University between May 2003 and November 2014 were retrospectively evaluated. The analyzed clinical factors included maximum tumor diameter, the R.E.N.A.L. Nephrometry Score, occurrence of ATI during surgery, and the postoperative pathological findings including the presence of a pseudocapsule. Port site metastasis, tumor seeding, and local recurrence were investigated by routine follow-up computed tomography during the postoperative period. Results: Among enrolled 156 procedures, 12 (7.7%) showed ATI during surgery. Positive surgical margin and local tumor recurrence were observed in 5 and 1 cases in the non-ATI group, respectively, as compared with in no cases in the ATI group. Port site metastasis or tumor seeding was not observed in either group. Multivariate analysis indicated that pseudocapsule formation significantly correlated with ATI (P = .022) and that maximum tumor diameter was a possible predictor of ATI (P = .054). Conclusion: To our knowledge, there are no previous studies to evaluate the impact of ATI, and we here, for the first time, report that the risk of ATI is influenced by the presence of a pseudocapsule, and, to some degree, by the tumor size. Moreover, we also show that ATI during laparoscopic partial nephrectomy is not necessarily associated with poor outcomes such as local tumor recurrence. (C) 2015 Elsevier Inc. All rights reserved.

DOI： 10.1016/j.clgc.2015.11.013

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：ELSEVIER SCIENCE INC  

Background The prevalence of, and mortality from, colorectal cancer is increasing worldwide, and new strategies for prevention are needed to reduce the burden of this disease. The oral diabetes medicine metformin might have chemopreventive effects against cancer, including colorectal cancer. However, no clinical trial data exist for the use of metformin for colorectal cancer chemoprevention. Therefore, we devised a 1-year clinical trial to assess the safety and chemopreventive effects of metformin on sporadic colorectal cancer (assessed by adenoma and polyp recurrence) in patients with a high risk of adenoma recurrence.
Methods This trial was a multicentre, double-blind, placebo-controlled, randomised phase 3 trial. Non-diabetic adult patients who had previously had single or multiple colorectal adenomas or polyps resected by endoscopy were enrolled into the study from five hospitals in Japan. Eligible patients were randomly assigned (1:1) to receive oral metformin (250 mg daily) or identical placebo tablets by a stratified computer-based randomisation method, with stratification by institute, age, sex, and body-mass index. All patients, endoscopists, doctors, and investigators were masked to drug allocation until the end of the trial. After 1 year of administration of metformin or placebo, colonoscopies were done to assess the co-primary endpoints: the number and prevalence of adenomas or polyps. Our analysis included all participants who underwent random allocation, according to the intention-to-treat principle. This trial is registered with University Hospital Medical Information Network (UMIN), number UMIN000006254.
Findings Between Sept 1, 2011, and Dec 30, 2014, 498 patients who had had single or multiple colorectal adenomas resected by endoscopy were enrolled into the study. After exclusions for ineligibility, 151 patients underwent randomisation: 79 were assigned to the metformin group and 72 to the placebo group. 71 patients in the metformin group and 62 in the placebo group underwent 1-year follow-up colonoscopy. The prevalence of total polyps (hyperplastic polyps plus adenomas) and of adenomas in the metformin group was significantly lower than that in the placebo group (total polyps: metformin group 27 [38.0%; 95% CI 26.7-49.3] of 71 patients, placebo group 35 [56.5%; 95% CI 44.1-68.8] of 62; p=0.034, risk ratio [RR] 0.67 [95% CI 0.47-0.97]; adenomas: metformin group 22 [30.6%; 95% CI 19.9-41.2] of 71 patients, placebo group 32 [51.6%; 95% CI 39,2-64.1] of 62; p=0.016, RR 0.60 [95% CI 0.39-0.92]). The median number of polyps was zero (IQR 0-1) in the metformin group and one (0-1) in the placebo group (p=0.041). The median number of adenomas was zero (0-1) in the metformin group and zero (0-1) in the placebo group (p=0.037). 15 (11%) of patients had adverse events, all of which were grade 1. We recorded no serious adverse events during the 1-year trial.
Interpretation The administration of low-dose metformin for 1 year to patients without diabetes was safe. Low-dose metformin reduced the prevalence and number of metachronous adenomas or polyps after polypectomy. Metformin has a potential role in the chemoprevention of colorectal cancer. However, further large, long-term trials are needed to provide definitive conclusions.

DOI： 10.1016/S1470-2045(15)00565-3

Web of Science

PubMed

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：神奈川県医師会  

researchmap

DOI： 10.1002/ccr3.401

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：E-CENTURY PUBLISHING CORP  

Background: Xp11.2 translocation-associated renal cell carcinomas (Xp11.2-RCCs) are a rare subtype of renal neoplasm. The incidence of these tumors is lower in the elderly than in children and young adults. Furthermore, there have been very few reports of Xp11.2-RCCs with multilocular cystic structures. Case: A 66-year-old Japanese female was admitted to our hospital with amultilocular cystic lesion in her right kidney. Computed tomography revealed that the lesion occupied the cortex and medulla of the upper pole of the right kidney, and partial nephrectomy was performed. Resected tissue contained a multilocular cystic tumor measuring 4.5x4.3x4.0 cm. Histologically, the cyst walls were composed of thin fibrous septa covered by a few layers of tumor cells with a clear cytoplasm. Psammoma bodies were scattered in the cyst lining. Immunohistochemically, most of tumor cells were positive for TFE3, alpha-methylacyl CoA racemase (AMCAR), and cathepsin K. Tumor cells also showed immunoreactivities for CD10 and cytokeratins, but were negative for carbonic anhydrase 9 and c-kit. Fluorescence in situ hybridization using a break-apart probe revealed monoalleleic TFE3 breakage. Based on these results, the tumor was diagnosed as Xp11.2-RCC, even though the patient's age and morphological architecture were not representative. Conclusion: This case demonstrated that Xp11.2-RCCs may occur in adults with multilocular cystic structures. Retrospectively, the recognition of psammoma bodies represented an important diagnostic sign.

Web of Science

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：JAPAN SOC INTERNAL MEDICINE  

Multifocal fibrosclerosis (MFS), which causes systemic and chronic connective tissue inflammation, has been associated with IgG4 and regarded as an identical entity with "IgG4-related disease (IgG4-RD)". Although a few cases of MFS mimicking IgG4-RD histopathologically, despite the absence of a serum IgG4 elevation and IgG4-positive plasma cell infiltration, have been reported, there is, so far, little information regarding such exceptional cases. We herein demonstrate a case of non-IgG4-related MFS presenting with periaortitis and parotiditis, whose histological findings were consistent with IgG4-RD despite the absence of elevated serum and tissue IgG4 levels.

DOI： 10.2169/internalmedicine.55.6297

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：HINDAWI PUBLISHING CORP  

We determined whether prostate specific antigen (PSA) would decrease with immediate antiandrogen switching from bicalutamide (BCL) to flutamide (FLT) in patients receiving combined androgen blockade for advanced prostate cancer. From 2002 to 2006, 20 patients who showed PSA failure after first-line hormonal therapy with a luteinizing hormone-release hormone (LH-RH) agonist and BCL were enrolled. All patients were immediately switched from BCL to FLT, administered with an LH-RH agonist, as second-line combined androgen blockade (CAB). We evaluated the PSA response to second-line CAB. Eight patients (40%) were responsive, showing PSA decreases of at least 50%. The median (range) duration of the PSA response was 18.4 (3-26) months. Second-line CAB using FLT was effective in 40% of patients who received first-line CAB using BCL. The lower Gleason scores at the initial prostate biopsy probably reflect the response to second-line CAB. Responders showed significantly better OS and CSS in the determination of any PSA decline and 40% PSA decline. The median OS duration in nonresponders and responders (40% PSA decline) was 1433 days versus 3617 days. It is concluded that an immediate switch from BCL to FLT is effective for some CRPC patients after first-line CAB using BCL.

DOI： 10.1155/2016/4083183

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：WILEY  

Aims: High-grade fetal adenocarcinoma (H-FLAC) is a rare variant of pulmonary adenocarcinoma; this study aims to elucidate its clinicopathological features and genetic abnormalities.
Methods and results: Clinicopathological, immunohistochemical and mutational analyses were performed on 20 surgically resected lung cancers that showed H-FLAC histology in various proportions. These tumours predominantly occurred in elderly males and in 10 patients who were heavy smokers. Four cases were pure H-FLAC, and 16 cases were mixed H-FLAC, which were found to be combined with conventional-type adenocarcinoma (15 cases), large-cell neuroendocrine carcinoma (three cases), small-cell carcinoma (one case), enteric adenocarcinoma (two cases), choriocarcinoma (two cases), and a solid-clear cell pattern (seven cases). The fetal phenotype and diverse differentiation were supported by the immuno-expression of a-fetoprotein (95%), thyroid transcription factor-1 (TTF-1) (50%), neuroendocrine markers (30-45%), proneural markers (50-69%), and CDX2 (40%). Except for TTF-1 expression (pure H-FLACs, 0%; mixed H-FLACs, 63%), there were no significant differences in histological or immunohistochemical findings between pure and mixed H-FLACs. EGFR, KRAS, BRAF and PIK3CA mutations were identified in 20%, 0%, 0% and 7% of the tumours, respectively.
Conclusions: Lung adenocarcinomas with H-FLAC features possess the potential for multidirectional differentiation, and are not strongly associated with known major driver gene mutations.

DOI： 10.1111/his.12711

Web of Science

PubMed

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：英語   出版者・発行元：日本癌学会  

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：SPRINGER JAPAN KK  

Morphological detection of cancer cells in the rabbit VX2 allograft transplantation model is often difficult in a certain region such as serosal cavity where reactive mesothelial cells mimic cancer cells and both cells share common markers such as cytokeratins. Therefore, tagging VX2 cells with a specific and sensitive marker that easily distinguishes them from other cells would be advantageous. Thus, we tried to establish a successively transplantable, enhanced green fluorescent protein (EGFP)-expressing VX2 model. Cancer cells obtained from a conventional VX2-bearing rabbit were cultured in vitro and transfected with an EGFP-encoding vector, and then successively transplanted in Healthy Japanese White rabbits (HJWRs) (n = 8). Besides, conventional VX2 cells were transplanted in other HJWRs (n = 8). Clinicopathological comparison analyses were performed between the two groups. The success rate of transplantation was 100 % for both groups. The sensitivity and specificity of EGFP for immunohistochemical detection of VX2 cells were 84.3 and 100 %, respectively. No significant differences in cancer cell morphology, tumor size (P = 0.742), Ki-67 labeling index (P = 0.878), or survival rate (P = 0.592) were observed between the two. VX2 cells can be genetically altered, visualized by EGFP, and successively transplanted without significant alteration of morphological and biological properties compared to those of the conventional model.

DOI： 10.1007/s00795-014-0071-2

Web of Science

PubMed

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本小児血液・がん学会  

researchmap

記述言語：日本語   出版者・発行元：神奈川県医師会  

researchmap

記述言語：英語   出版者・発行元：AVES  

DOI： 10.5152/tjg.2014.4114

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

High serum calcium (Ca) due to aberrant secretion of tumor parathyroid hormone-like hormone (PTHLH) is a well-known paraneoplastic sign and is associated with poor prognosis in patients with renal cell carcinoma (RCC). However, the status of serum Ca and tumor PTHLH expression have not been verified using the 2004 World Health Organization (WHO) renal tumor classification. We retrospectively reviewed corrected serum Ca levels at initial onset (n = 683) and/or as of recurrence (n = 71) in patients with RCC. We also examined a total of 623 renal parenchymal tumor samples for PTHLH mRNA expressions by quantitative real-time PCR. High serum Ca concomitant with PTHLH overexpression in tumors was observed exclusively in clear cell RCC but not in other non clear cell subtype tumors, including papillary, chromophobe, collecting-duct, unclassified, and other rare subtype RCCs or in benign oncocytomas and angiomyolipomas. In clear cell RCC, PTHLH expression was significantly high in male patients, and was associated with a symptomatic presentation, higher grade, and higher stage cases, whereas it was not associated with VHL gene status. Univariate analyses demonstrated that high PTHLH expression was strongly associated with poor outcome both in overall survival (OS) and disease-free survival (DFS) for patients who underwent standard nephrectomy. Further multivariate Cox analyses revealed that the PTHLH expressions remained as independent prognostic parameters for OS but not for DFS. These data suggest that the previously characterized tumor signatures of high serum Ca due to high PTHLH expression and poor prognosis are clear cell RCC-specific features, whereas these characteristics are rare in non clear cell RCCs.

DOI： 10.1002/cam4.270

PubMed

researchmap

記述言語：日本語   出版者・発行元：神奈川県医師会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

扁桃に発生した濾胞樹状細胞肉腫(Follicular dendritic cell sarcoma:FDCS)の1例を経験したので報告する。患者は80代男性、歯科で中咽頭腫瘤を指摘され前医受診。前医施行の生検では組織型確定には至らなかったが、悪性腫瘍が疑われ、手術目的で当院耳鼻咽喉科紹介受診、拡大扁摘術施行。組織学的には卵円形〜紡錘形細胞が渦巻様配列を伴って充実性に増殖する像を認めた。免疫染色ではCD21、CD23、CD35が陽性であり、HE染色所見と併せて濾胞樹状細胞肉腫と診断した。(著者抄録)

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   掲載種別：研究論文（学術雑誌）   出版者・発行元：Kyushu University  

Fibrous hamartoma of infancy (FHI) is a relatively rare subcutaneous soft tissue tumor in children. We report on a 1-year-old girl with a fibrous hamartoma on the right upper arm. The subcutaneous tumor was first noticed at the age of 5 months and had grown gradually. When the patient was 11 months old, hypertrichosis was noticed on the tumor surface. The tumor had a diameter of around 17 mm and was elastic hard. The surface of the mass was smooth, and its movability was good. The surgical resection was performed when the girl was 12 month old. Histopathologically, an organoid structure was apparent, consisting of mature fat tissue and complicating fibrous trabeculae, as well as an increased concentration of immature mesenchymal cells. The histological diagnosis was fibrous hamartoma of infancy (FHI). Five years and nine months after the operation, no recurrence has been noticed. We suggest that FHI should be included in a differential diagnosis of subcutaneous tumors in infants.

DOI： 10.2336/nishinihonhifu.76.92

Scopus

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：S. Karger AG  

Objective: This study aimed to evaluate the usability of peritoneal swabbing cytology (PSC) with cotton swabs as an effective method for the detection of peritoneal metastasis during the staging laparotomy of epithelial ovarian carcinomas and borderline tumours (EOC/BT). Study Design: One hundred and ninety-two patients with EOC/BT who underwent laparotomy at the Yokohama City University Hospital from 2004 to 2010 were analysed retrospectively. The positive rates of the PSC and ascites/peritoneal-wash cytology (A/PWC) were compared. Results and Conclusion: Both A/PWC and PSC were performed during laparotomy (n = 140). The rate of metastasis detection with A/PWC was significantly higher than with PSC (50.0 vs. 20.7%, p &lt
0.001 by χ2 test). The superior detection ability of A/PWC was observed regardless of the presence of macroscopic peritoneal metastasis in 56 cases with macroscopic peritoneal metastasis, with A/PWC detecting 80.3% of cases versus PSC detecting 37.5% (p = 0.013). In 84 cases without macroscopic peritoneal metastasis, A/PWC detected 29.8% of cases versus 9.5% with PSC (p = 0.003). Our results suggest that PSC is insufficient to evaluate the peritoneal metastasis for the surgical staging of EOC/BT. Other procedures such as subdiaphragmatic A/PWC should be included when possible to maximize the accuracy of diagnosis. © 2014 S. Karger AG, Basel.

DOI： 10.1159/000360412

Scopus

PubMed

researchmap

記述言語：日本語   出版者・発行元：(一社)日本胆道学会  

症例は70歳女性。USで肝内胆管、総胆管の拡張を認めた。CTで下部胆管に造影効果を有する腫瘍を認めた。内視鏡で十二指腸乳頭部の口側隆起は球状に腫大し、開口部粘膜の潰瘍性病変と十二指腸粘膜の襞集中を認めた。胆管深部挿管は困難でありsuprapapillary incisionを施行した。ERCで胆管末端の嚢腫状拡張を認めた。EUS、IDUSでは胆管末端は12mm径の嚢腫状の拡張を認め、十二指腸乳頭部に腫瘍の筋層浸潤を認めた。乳頭部生検は腺癌であった。術前画像診断でAbから発生した浸潤癌と診断した。非共通管拡張型のCholedochoceleの合併も示唆された。手術施行されたが開腹時、肝臓に微小結節が多発していたため試験開腹のみとなった。(著者抄録)

researchmap

その他リンク： https://search.jamas.or.jp/default/link?pub_year=2013&ichushi_jid=J02149&link_issn=&doc_id=20130607100012&doc_link_id=%2Fdw1tando%2F2013%2F002702%2F012%2F0240-0246%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdw1tando%2F2013%2F002702%2F012%2F0240-0246%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Endocrine mucin-producing sweat gland carcinoma (EMPSGC) has recently been recognised as a low-grade carcinoma that almost always occurs on the eyelid. This carcinoma is very rare, with only 20 cases (including the present one) having been reported in the literature. EMPSGC is frequently found in association with invasive mucinous carcinoma. While EMPSGC treatments consist of a complete surgical removal, there has been no consensus regarding the surgical margin. Therefore, reports on surgical management of EMPSGC may potentially provide important therapeutic information. Here, we present a case of a 74-year-old man with EMPSGC of the eyelid that repeatedly recurred despite surgical treatments at another institution. After referral to our department, the patient underwent tumour excision. However, the specimen revealed a positive surgical margin and thus, he subsequently underwent a wider excision. There has been no sign of tumour recurrence or metastasis 6 months after his last operation. This article reviews the current literature and discusses the surgical management of EMPSGC. © 2012 The Authors. Australasian Journal of Dermatology © 2012 The Australasian College of Dermatologists.

DOI： 10.1111/j.1440-0960.2011.00857.x

Scopus

PubMed

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.3174/ajnr.A3159

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：Elsevier Inc.  

DOI： 10.1016/j.gynor.2013.02.005

Scopus

PubMed

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：BIOMED CENTRAL LTD  

Background: Colorectal cancer is one of the major neoplasms and a leading cause of cancer death worldwide, and new preventive strategies are needed to lower the burden of this disease. Metformin, a biguanide, which is widely used for treating diabetes mellitus, has recently been suggestive to have a suppressive effect on tumorigenesis and cancer cell growth. In a previous study conducted in non-diabetic subjects, we showed that oral short-term low-dose metformin suppressed the development of colorectal aberrant crypt foci (ACF). ACF have been considered as a useful surrogate biomarker of CRC, although the biological significance of these lesions remains controversial. We devised a prospective randomized controlled trial to evaluate the chemopreventive effect of metformin against metachronous colorectal polyps and the safety of this drug in non-diabetic post-polypectomy patients.
Methods/Design: This study is a multi-center, double-blind, placebo-controlled, randomized controlled trial to be conducted in non-diabetic patients with a recent history of undergoing colorectal polypectomy. All adult patients visiting the Yokohama City University hospital or affiliated hospitals for polypectomy shall be recruited for the study. Eligible patients will then be allocated randomly into either one of two groups: the metformin group and the placebo group. Patients in the metformin group shall receive oral metformin at 250 mg per day, and those in the placebo group shall receive an oral placebo tablet. At the end of 1 year of administration of metformin/placebo, colonoscopy will be performed to evaluate the polyp formation.
Discussion: This is the first study proposed to explore the effect of metformin against colorectal polyp formation. Metformin activates AMPK, which inhibits the mammalian target of rapamycin (mTOR) pathway. The mTOR pathway plays an important role in the cellular protein translational machinery and cell proliferation. Patients with type 2 diabetes taking under treatment with metformin have been reported to be at a lower risk of cancer development than those not taking under treatment with metformin. We showed in a previous study that metformin suppressed the formation of human colorectal ACF. We therefore decided to conduct a study to determine whether metformin might suppress the formation of human colorectal polyps.
Trial registration: This trial has been registered in the University hospital Medical Information Network (UMIN) Clinical Trials Registry as UMIN000006254

DOI： 10.1186/1471-2407-12-118

Web of Science

PubMed

researchmap

記述言語：日本語   出版者・発行元：(公社)日本小児科学会  

researchmap

記述言語：日本語   掲載種別：研究論文（学術雑誌）  

A retroperitoneal angioleiomyoma was incidentally detected in a 56-year-old woman during an examination of cardiovascular disease, and referred to the department of urology. Computed tomography (CT) showed a solid tumor approximately 3 cm in diameter, enhanced heterogeneously adjacent to the right adrenal and renal vein on magnetic resonance imaging the tumor showed a low intensity in the Tl-weighted image and high intensity in T2-weighted image. These radiographic findings suggested a retroperitoneal tumor such as paraganglioma, angioma. Furthermore, because she was a carrier of Human Adult T Cell Leukemia Virus-I (HTLV-I) this tumor was suspected to have relevance to malignant lymphoma. We performed laparoscopic surgical excision of the tumor. Pathlogical diagnosis was an angioleiomyoma. Angioleiomyoma is a rare type of leiomyoma originating from smooth muscle and containing thick-walled vessels. Only a few cases of retroperitoneal angioleiomyoma have been reported.

Scopus

PubMed

researchmap

記述言語：日本語   出版者・発行元：神奈川県医師会  

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：SPRINGER  

The aim of this study is to evaluate the potential and limitation of FDG-PET/CT for detecting prostate cancer in subjects with an elevated serum prostate-specific antigen (PSA) level. Although [(18)F]-2-fluoro-2-deoxyglucose positron emission tomography (FDG-PET) has limited value in detecting prostate cancer, the potential of PET/CT has not been precisely evaluated, since positron emission tomography/computed tomography (PET/CT) provides accurate localization of functional findings obtained by PET.
Subjects with an increasing PSA level suggestive of prostate cancer were enrolled in this study. FDG-PET/CT was performed prior to prostate biopsy and the findings were compared with the pathological results.
Fifty subjects with an elevated serum PSA level took part in this study. The sensitivity, specificity and positive predictive value (PPV) of FDG-PET/CT in the prostate were 51.9% (27/52 areas), 75.7% (112/148 areas) and 42.9% (27/63 areas), respectively; those in the peripheral zone were 73.3% (22/30 areas), 64.3% (45/70 areas) and 46.8% (22/47 areas), respectively; and those in the central gland were 22.7% (5/22 areas), 85.9% (67/78 areas) and 31.3% (5/16 areas), respectively. The estimated cut-off values according to the highest odds ratio (OR) were age of 70 years [OR: 7.00, 95% confidence interval (CI): 1.89-25.93] and a PSA value of 12.0 ng/ml (OR: 10.77, 95% CI: 2.78-41.74). The FDG-PET/CT could potentially detect cancer with 80.0% sensitivity and 87.0% PPV in cases with a Gleason score of 7 or greater.
FDG-PET/CT was appropriate for detecting peripheral zone prostate cancer in patients at more than an intermediate risk.

DOI： 10.1007/s12149-010-0424-4

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：JAPAN SOC INTERNAL MEDICINE  

We report on a 60-year-old man, having angioimmunoblastic T-cell lymphoma (AITL) with aberrant expression of CD20. The initial biopsy specimen showed features of AITL with the typical CD3(+)CD20(-) immunophenotype. As the disease progressed, biopsy specimens of the recurrent lesions showed unusual results for AITL with the CD20(+) immunophenotype in both flow cytometry and immunohistochemistry analysis. Double immunostaining confirmed that the lymphoma cells were simultaneously positive for CD3 and CD20. The disease became resistant to combined chemotherapies and the patient died 3 years after the initial diagnosis. Autopsy revealed a composite of AITL and EBV-positive diffuse large B-cell lymphoma. Although it is unknown whether the instability of the CD20 antigen expression is correlated with the behaviour of AITL, the administration of rituximab should be considered as an alternative therapeutic option in such cases.

DOI： 10.2169/internalmedicine.50.4386

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Abstract. Introduction. Adrenal pseudocysts are rare cystic masses that arise from the adrenal gland and which are usually non-functional and asymptomatic. Adrenal pseudocysts consist of a fibrous wall without an epithelial or endothelial lining. We report the case of a patient with a giant adrenal pseudocyst presenting with right hypochondralgia and high fever. Case presentation. A 52-year-old Japanese man was admitted with right hypochondralgia and a chill. Abdominal computed tomography revealed a well-defined cystic mass measuring 19 cm which was located in the right adrenal region and the contents of which were not enhanced with contrast medium. Abdominal ultrasonography revealed a heterogeneously hypo-echoic lesion with a peripheral high-echoic rim. Serum hormonal levels were almost normal. Despite treatment with antibiotics, the high fever persisted. Based on these findings, we made a preoperative diagnosis of a right adrenal cyst with infection. However, the possibility of malignancy still remained. The patient underwent laparotomy and right adrenal cyst excision with partial hepatectomy in order to relieve the symptoms and to confirm an accurate diagnosis. Histological examination revealed an adrenal pseudocyst with infection. His condition improved soon after the operation. Conclusion: We report a case of a giant adrenal pseudocyst with infection. Surgery is required for symptomatic cases in order to relieve the symptoms and in cases of uncertain diagnosis. © 2011 Momiyama et al
licensee BioMed Central Ltd.

DOI： 10.1186/1752-1947-5-135

Scopus

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：SPRINGER TOKYO  

Sebaceous carcinoma is the rarest type of oral malignancies. We report a case of primary sebaceous carcinoma of the tongue. Systemic imaging studies revealed that the patient had a T2N2cM0 (International Union Against Cancer guidelines) primary lingual tumor. Histopathological examination revealed neoplastic sebocytic and basaloid cells, and Sudan III staining and electron microscopy revealed intracytoplasmic lipid droplets. The neoplastic cells stained positive for adipophilin; epithelial membrane antigen; epithelial antigen; and cytokeratins 7, 8, and 15, but negative for cytokeratins 5/6, 18, 19, and 20; the androgen receptor; and carcinoembryonic antigen. Superselective intraarterial chemotherapy was administered via the superficial temporal artery concurrent with daily radiotherapy. Multiple biopsies confirmed a complete response of the primary lesion. The patient then underwent neck dissection followed by pathological examination, which revealed lymph nodes metastases. After postoperative radiotherapy to the neck, distant metastases were identified in the mediastinal lymph nodes and the lung. The patient died 17 months after completing the initial course of chemoradiotherapy. Our case demonstrates that superselective intraarterial chemotherapy combined with concurrent radiotherapy can be effective in treating the primary lesion of patients with a sebaceous carcinoma of the tongue. However, an effective strategy to eradicate metastases has yet to be established.

DOI： 10.1007/s00795-010-0521-4

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：OXFORD UNIV PRESS  

Background. A large body of accumulated data has now revealed that podocytes play a major role in the development of proteinuria. However, the mechanisms of podocyte injury, leading to foot process effacement and proteinuria, are still unclear partly due to the current lack of an appropriate strategy for preparing podocytes. In this study, we have developed a novel method of rapid isolation of podocytes from mice using magnetic activated cell sorting with an anti-nephrin antibody.
Methods. After endothelial cell depletion using anti-CD31 antibody, nephrin-positive cells were prepared from mouse kidneys using magnetic activated cell sorting with polyclonal rabbit anti-nephrin antibody. Purity of the positively sorted cells was determined by confocal microscopy and fluorescence-activated cell sorting (FACS) analysis. Expression profiles of podocyte-specific molecules in the sorted fractions were characterized by qualitative PCR and immunoblot analysis.
Results. Nephrin-positive cells, isolated from mouse kidneys within 6 h, showed dual positivity for synaptopodin and rabbit IgG on confocal microscopy. FACS analysis revealed that the purity of the positively sorted fractions was similar to 75%. The nephrin-positive cells sorted by this approach showed a significantly higher expression of podocyte-specific molecules compared with nephrin-negative fractions.
Conclusions. These data strongly suggest that our novel method for isolating podocytes has great utility for various downstream applications such as genomic analysis, proteomics and transcriptomics to elucidate molecular profiling of podocyte biology in vivo compared with conventional methods as our approach requires only several hours to complete and no tissue culture.

DOI： 10.1093/ndt/gfq323

Web of Science

PubMed

researchmap

記述言語：英語   出版者・発行元：WILEY-BLACKWELL PUBLISHING, INC  

DOI： 10.1111/j.1365-2303.2010.00736.x

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：SPRINGER  

Paraneoplastic neurological syndromes (PNSs) are a heterogeneous group of neurological disorders caused by immune-mediated mechanisms. The incidence of PNS is much less than 1% for solid tumors, except for small-cell lung cancer and thymoma. We report a rare case of gastric cancer that presented with primary clinical findings of PNS. The patient was a 63-year-old woman who was admitted for worsening neuropathy. Laboratory and neurological tests excluded a nutritional deficit, diabetes mellitus, and connective tissue disease as causes of her neuropathy. Computed tomography (CT) of the abdomen, positron emission tomography (PET)-CT, and endoscopy of the stomach revealed gastric cancer with lymph node swelling. Distal gastrectomy was performed and pathological and immunohistochemical examinations indicated endocrine cell carcinoma. The gastrectomy stopped the exacerbation of her symptoms and recurrence was not observed, but the neurological disorders were irreversible. This case suggests that early diagnosis of the primary tumor is required to improve the outcome in patients with PNS.

DOI： 10.1007/s10120-010-0563-2

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：PUBLIC LIBRARY SCIENCE  

Background: Sandhoff disease is a lysosomal storage disorder characterized by the absence of beta-hexosaminidase and storage of GM2 ganglioside and related glycolipids. We have previously found that the progressive neurologic disease induced in Hexb(-/-) mice, an animal model for Sandhoff disease, is associated with the production of pathogenic anti-glycolipid autoantibodies.
Methodology/Principal Findings: In our current study, we report on the alterations in the thymus during the development of mild to severe progressive neurologic disease. The thymus from Hexb(-/-) mice of greater than 15 weeks of age showed a marked decrease in the percentage of immature CD4(+)/CD8(+) T cells and a significantly increased number of CD4(+)/CD8(-) T cells. During involution, the levels of both apoptotic thymic cells and IgG deposits to T cells were found to have increased, whilst swollen macrophages were prominently observed, particularly in the cortex. We employed cDNA microarray analysis to monitor gene expression during the involution process and found that genes associated with the immune responses were upregulated, particularly those expressed in macrophages. CXCL13 was one of these upregulated genes and is expressed specifically in the thymus. B1 cells were also found to have increased in the thy mus. It is significant that these alterations in the thymus were reduced in FcR gamma additionally disrupted Hexb(-/-) mice.
Conclusions/Significance: These results suggest that the FcR gamma chain may render the usually poorly immunogenic thymus into an organ prone to autoimmune responses, including the chemotaxis of B1 cells toward CXCL13.

DOI： 10.1371/journal.pone.0012105

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

INTRODUCTION: Standard endoscopic mucosal resection or endoscopic submucosal dissection is a procedure for patients with minute cancers, complicated with esophageal varices that puts them at high risk of bleeding. CASE PRESENTATION: We present the case of a 77-year-old Japanese man with alcoholic cirrhosis who underwent a routine endoscopy examination as a screening procedure for esophageal varices and was incidentally diagnosed as having minute cancer of the esophagogastric junction with esophageal varices. Endoscopic ultrasonography findings suggested that the minute cancer was a non-invasive carcinoma (carcinoma in situ) and a 2 mm in diameter blood vessel, feeding the esophageal varices, pierced the lesion. Following the examination, we carried out endoscopic treatment of the minute cancer and esophageal varices. Endoscopic variceal ligation was performed using a pneumo-activated device (Sumitomo Bakelite, Tokyo, Japan). Two years after the treatment, during the follow-up endoscopic examination on the patient, recurrence of carcinoma was not detected endoscopically or histologically. CONCLUSION: Endoscopic therapy using an endoscopic variceal ligation device for minute cancer of the esophagogastric junction, complicated with esophageal varices, may be an acceptable and easily applicable method.

DOI： 10.1186/1752-1947-4-149

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：SPANDIDOS PUBL LTD  

Claudin-7 is a tight junction protein that plays an important role in tumorigenesis, tumor invasion and metastasis We examined the clinical significance of claudin-7 expression in 75 postsurgical non-small cell lung cancer (NSCLC) patients Claudin-7 expression was measured immunohistochemically and was found to be high in 25 patients (33 3%) and low in 50 (66 7%) Survival was significantly poorer in patients with claudin-7-low than in those with claudin-7-high NSCLCs (P=0 024) In particular, survival was significantly poorer in patients with claudin-7-low than in those with claudin-7-high squamous cell carcinomas (P=0 011) A reduced expression of claudin-7 was associated with poor outcome in NSCLCs Claudin-7 may thus be a useful biomarker and a potential therapeutic target in patients with NSCLC

DOI： 10.3892/ol_00000088

Web of Science

researchmap

記述言語：日本語   出版者・発行元：Japanese College of Surgeons  

近年，カルチノイドと大腸癌の重複症例の報告は増加しているものの，その数はいまだ少ない．今回，われわれは直腸カルチノイドに対して内視鏡的切除を行い，S状結腸進行癌に対しては根治的切除術を施行した同時性重複症例を経験したので報告する．<BR> 症例は79歳の男性．既往に高血圧，気管支喘息，心房細動，糖尿病を併存し加療中．2007年10月便潜血陽性を指摘され，11月前医で下部消化管内視鏡検査を施行し肛門縁から25cmのS状結腸に２型腫瘍を，5cmの直腸（Rb）に径1.5cmのIsp型腫瘍を認めた．ポリペクトミーを施行しカルチノイドと診断された．CT検査では肝肺転移はなく，直腸近傍にリンパ節腫大はなかった．翌年１月当科受診．２月S状結腸切除術を施行し，併存症が多く高リスク症例のため郭清はD2とした．病理組織所見はS，tub2，2型，se，n1，fStage IIIa，根治度Aであった．術後経過は順調で術後13日で退院となり，25カ月経過した現在無再発生存中である．

DOI： 10.4030/jjcs.35.193

CiNii Books

researchmap

その他リンク： http://search.jamas.or.jp/link/ui/2010294674

記述言語：日本語   出版者・発行元：日本臨床外科学会  

researchmap

記述言語：日本語   出版者・発行元：医学図書出版(株)  

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

The case was a 67-year-old male who visited our hospital with a major complaint of macroscopic hematuria. A bladder tumor was found. When a transurethral resection of the bladder tumor was performed, the histopathological diagnosis was neuroendocrine bladder cancer. After chemotherapy with cisplatin and etoposide a partial shrinkage of the tumor was observed
however, the patient expired 7 months after the first visit. Copyright © 2010 S. Karger AG.

DOI： 10.1159/000289584

Scopus

PubMed

researchmap

記述言語：日本語   出版者・発行元：日本臨床外科学会  

researchmap

記述言語：英語   出版者・発行元：LIPPINCOTT WILLIAMS & WILKINS  

We report the F-18 FDG PET appearance of an adult prostatic sarcoma. A 76-year-old man with a history of prostate cancer treated by hormone therapy, a lobulated heterogeneous mass in the prostate gland was found on a follow-up pelvic computed tomography. Although the prostate-specific antigen level was within the normal range, transperineal core needle biopsy was performed because recurrent prostatic carcinoma was suspected. The tumor was pathologically diagnosed as a prostatic sarcoma with no evidence of existing prostatic carcinoma. F-18 FDG-PET revealed avid-FDG uptake (maximum SUV of 7.1) and no evidence of distant metastasis.

DOI： 10.1097/RLU.0b013e3181a34681

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：AMER MEDICAL ASSOC  

Sinonasal malignant tumors constitute less than 1% of all neoplasms and approximately 3% of those of the upper aerodigestive tract.(1) Sinonasal malignant neoplasms most commonly affect the maxillary sinuses (approximately 60%), followed by the nasal cavity (approximately 22%), ethmoidal sinus (approximately 15%), and frontal and sphenoid sinuses (&lt;3%). 1 Sinonasal neoplasms are diverse, with most being squamous cell carcinoma or variants (55%), followed by nonepithelial neoplasms (20%), glandular tumors (15%), undifferentiated carcinoma (7%), and miscellaneous tumors (3%).(1) We describe an exceedingly unusual case of ethmoidal lipid-rich carcinoma with focal glandular structures. To our knowledge, such a case has not been documented in the literature to date.

DOI： 10.1001/archoto.2009.27

Web of Science

PubMed

researchmap

記述言語：英語   出版者・発行元：The Japanese Society for Lymphoreticular Tissue Research  

There are several reports describing [<SUP>18</SUP>F] fluorodeoxyglucose positron emission tomography (FDG-PET) findings in patients with lymphomatoid granulomatosis (LYG). We report a case of grade I LYG that showed increased uptake of FDG. The patient was a 63-year-old Japanese male who underwent an FDG-PET/computed tomography (CT) scan in screening for a malignant lesion. Increased uptake of FDG [maximum standard uptake value (SUV<SUB>max</SUB>), 3.7] was observed in the right hilar region in FDG-PET and enhanced CT revealed a round, abnormal mass that also showed increased FDG uptake. The patient had no previous symptoms. A tumor biopsy was performed and the histological diagnosis was grade I LYG. Therefore, increased SUV<SUB>max</SUB> in FDG-PET might be useful for diagnosing of LYG. [<I>J Clin Exp Hematopathol 49(1) : 39-44, 2009</I>]

DOI： 10.3960/jslrt.49.39

PubMed

CiNii Books

researchmap

その他リンク： http://search.jamas.or.jp/link/ui/2009344729

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：WILEY-BLACKWELL PUBLISHING, INC  

Epithelioid angiomyolipoma (eAMLoma) is an uncommon renal mesenchymal tumor with malignant potential and is frequently associated with tuberous sclerosis (TSC). It is composed of polygonal large-sized tumor cells arranged in an epithelioid manner. Differential diagnosis from renal cell carcinoma (RCC) is often challenging because of its epithelioid morphology. Herein is reported three cases of eAMLoma, involving one in a 28-year-old man with TSC and two in women without TSC (34 and 62 years of age, respectively). The male TSC patient had microscopic conventional AMLomas in the same kidney. All patients were positive for melanoma (reactive with HMB45 antibody, and positive for melan A, tyrosinase and microphthalmia transcription factor) and smooth muscle markers (positive for alpha-smooth muscle-specific actin), but not for epithelial markers (cytokeratin, epithelial membrane antigen). In particular, the translocation RCC is an important differential diagnostic candidate, in terms of the positive reaction with HMB45 and morphological similarity. The present tumor samples did not show any reactivity for transcription factor binding to IGHM enhancer 3 or transcription factor EB, which excluded the possibility of translocation RCC. The possibility of eAMLoma should be evaluated as a diagnostic candidate, especially in cases of renal tumors (i) in young patients; (ii) associated with TSC; or (iii) with an epithelioid morphology and a high nuclear grade.

DOI： 10.1111/j.1440-1827.2008.02322.x

Web of Science

PubMed

researchmap

記述言語：英語   出版者・発行元：JAPAN SOC INTERNAL MEDICINE  

DOI： 10.2169/internalmedicine.48.2229

Web of Science

PubMed

researchmap

記述言語：英語   出版者・発行元：JAPAN SOC INTERNAL MEDICINE  

DOI： 10.2169/internalmedicine.48.2823

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Introduction: The granulocyte-colony stimulating factor-producing tumor was first reported in 1977, however, anaplastic pleomorphic type carcinoma of the pancreas producing granulocyte-colony stimulating factor is still rare. Case presentation: A 63-year-old man was admitted to our hospital with body weight loss (-10 kg during months) and upper abdominal pain from 3 weeks. Abdominal computed tomography demonstrated a pancreatic tumor 10 cm in size and multiple low-density areas in the liver. On admission, the peripheral leukocyte count was elevated to 91,500/mm3 and the serum concentration of granulocyte-colony stimulating factor was 134 pg/mL (normal, &lt
18.1 pg/mL). Based on liver biopsy findings, the tumor was classified as an anaplastic pleomorphic-type carcinoma. Immunohistochemical staining showed that pancreatic carcinoma cells were positive for granulocyte-colony stimulating factor. The patient developed interstitial pneumonia, probably caused by granulocyte-colony stimulating factor, and died 11 days after admission. Conclusion: This is a rare case report of anaplastic pleomorphic-type carcinoma of the pancreas producing granulocyte-colony stimulating factor and confirmed by immunohistochemistry. © 2008 Nakajima et al
licensee BioMed Central Ltd.

DOI： 10.1186/1752-1947-2-391

Scopus

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：WILEY-BLACKWELL PUBLISHING, INC  

Herein is reported a case of mucin-producing carcinoma of the gallbladder in a 55-year-old Japanese woman. Although the patient&apos;s status and laboratory data initially suggested biliary pancreatitis due to gallstone, radiography and endoscopy confirmed the presence of pancreaticobiliary maljunction and a gallbladder tumor with excessive mucin, in which the duodenal papilla and the common bile duct were impacted. Following surgery, the gallbladder tumor was histopathologically diagnosed as a mixed endocrine-exocrine carcinoma. The carcinoma predominantly consisted of papillary, but also contained some tubular adenocarcinomatous components. Additionally, small foci of small cell and large cell neuroendocrine carcinomatous components were observed. There was no evidence of lymph node metastasis, distant metastasis, or direct invasion outside the gallbladder. Thus, the final classification of pT2N0M0 stage II was given to this lesion, according to the Union Internationale Contre le Cancer guidelines. The postoperative course was uneventful, and the carcinoma had not recurred in the absence of chemoradiotherapy for a period of 20 months. Mucin-producing gallbladder carcinoma is a rare clinical condition that can occur in patients with pancreaticobiliary maljunction. Detailed investigation of this condition is important to develop and refine effective therapeutic strategies.

DOI： 10.1111/j.1440-1827.2008.02311.x

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：SPRINGER  

Adrenocortical carcinoma (ACC) in childhood is rare: in 2002, only three new cases were reported in Japan. Although there is no established therapeutic management system, margin-free complete surgical excision of the tumor is very important to a satisfactory outcome. We report a case of ACC with bilateral pulmonary metastasis in a 10-year-old boy. Preoperative chest computed tomography (CT) showed pulmonary metastasis with near-water density. The patient was treated successfully with complete surgical resection of the primary tumor and adjuvant chemotherapy, including mitotane. After bilateral lung surgery for the metastatic lesions, the plasma dehydroepiandrosterone sulfate level became negative.

DOI： 10.1007/s00595-008-3788-5

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：SPRINGER TOKYO  

We report a rare case of adenosquamous carcinoma of the pancreas associated with humoral hypercalcemia of malignancy (HHM) in which parathyroid hormone-related protein (PTH-rP) was identified as the causative factor of hypercalcemia. A 72-year-old Japanese man was admitted to our institution complaining of fever and abdominal pain. Abdominal computed tomography demonstrated a large tumor in the body of the pancreas, with multiple liver metastases. Both serum calcium and PTH-rP levels were elevated. No accumulation was observed on bone scan with technetium-99. The patient died of pneumonia 3 months after admission. Autopsy demonstrated that the neoplasm in the pancreas showed an abrupt histological transition from adenocarcinoma to squamous cell carcinoma. PTH-rP was identified in the primary pancreatic tumor cells by immunohistochemical examination and a reverse-transcription polymerase chain reaction (RTPCR) method. We concluded that PTH-rP was the causative factor of the HHM, based on the laboratory data, immunohistochemical examination, and messenger RNA (mRNA) expression. This is a very rare report of adenosquamous cell carcinoma of the pancreas associated with HHM.

DOI： 10.1007/s00534-007-1258-x

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：SPRINGER  

Lung cancer associated with Sweet's syndrome is extremely rare. There are only seven reports of such cases. As far as could be determined from a comprehensive search, there is no reported operative case of lung cancer with this syndrome in the world literature. A 75-year-old Japanese man was diagnosed as having Sweet's syndrome. A chest computed tomography (CT) scan to screen for malignant lesions associated with this syndrome revealed an abnormal shadow in the lung. Although [F-18]2-fluoro-2-deoxy-D-glucose positron emission tomography showed no abnormal uptake, lung cancer was most strongly suspected by chest CT. His erythema improved rapidly with steroid therapy and he underwent a segmentectomy (S-6) of the right lower lobe. A pathological examination revealed lung adenocarcinoma (pT1N0M0: Stage Ia). The patient was discharged from the hospital without any worsening of Sweet's syndrome. We herein report a first operative case of an early stage lung adenocarcinoma with this syndrome.

DOI： 10.1007/s00595-007-3683-5

Web of Science

PubMed

researchmap

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：LIPPINCOTT WILLIAMS & WILKINS  

Background. The clinical symptoms, histological findings, and treatments for antibody-mediated rejection (AMR), Which is the leading cause of graft loss in adult ABO-incompatible liver transplantation (ABO-I-LT), have rarely been discussed.
Methods. We performed adult living donor ABO-I-LT on six patients. We used anti-CD20 monoclonal antibody combined with plasma exchange preoperatively and intraportal or hepatic-arterial infusion, consisting of prostaglandin El, corticosteroids, and protease inhibitor postoperatively to prevent AMR. Splenectomy was performed in patients 1, 4, 5 and 6 but not in patients 2 and 3. Weekly liver biopsies were performed after ABO-I-LT. When severe AMR was diagnosed, we performed plasma exchange combined with gamma-globulin bolus infusion (PE+IVIG).
Results. In patients 1-3, severe jaundice, rapid decreases in platelet counts, and severe coagulopathy were observed in the early postoperative period. Liver biopsies sampled after the onset of these clinical findings were characterized by severe periportal and lobular hemorrhagic and neutrophil infiltration, suggesting that severe AMR occurred. However, after the initiation of PE+IVIG, AMR was remedied in all three patients. In patients 4-6, severe AMR was not observed. Mild AMR characterized by mild portal hemorrhagic infiltration was observed in patient 4, and moderate AMR characterized by moderate periportal and lobular hemorrhagic infiltration was observed in patient 6. Patients 4-6 did not require PE+IVIG and their clinical course was uneventful.
Conclusion. Given the experience of these six patients, we consider that AMR may be graded based on liver biopsy findings including hemorrhagic infiltration and neutrophil infiltration, as well as clinical findings. All six patients are currently doing well.

DOI： 10.1097/TP.0b013e31815e9672

Web of Science

PubMed

researchmap

記述言語：英語   出版者・発行元：KARGER  

DOI： 10.1159/000127417

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：KARGER  

Background/Aims: To investigate the effects of deficient degradation of glycolipids on the morphological appearance of all retinal cells in a murine model of G(M2) gangliosidosis (Sandhoff disease). Methods: The morphological appearance of the retina in Sandhoff mice at symptomatic stages (3 and 4 months of age) was examined by immunohistochemistry, lectin histochemistry and electron microscopy. Results: Under a light microscope, intense immunoreactivity for G(M2) ganglioside was observed in the ganglion cell, inner plexiform, and inner nuclear layers in the Sandhoff mice. The ganglion cell layers and retinal pigment epithelium in the Sandhoff mice were stained intensely with concanavalin A agglutinin and succinylated wheat germ agglutinin. Ultrastructural studies revealed numerous inclusions in the cytoplasm of retinal ganglion cells and other neuronal cells (particularly amacrine cells), whereas we failed to detect apparent involvement of photoreceptor cells. In addition to the cytoplasmic inclusions in the retinal neurons, vacuolation was evident in the retinal pigment epithelium. Conclusion: These findings suggest that neuronal cells and pigment epithelial cells are more vulnerable to the deficient ganglioside degradation than other retinal cells in Sandhoff mice. Copyright (C) 2008 S. Karger AG, Basel.

DOI： 10.1159/000127831

Web of Science

PubMed

researchmap

記述言語：英語   出版者・発行元：The Japanese Society for Lymphoreticular Tissue Research  

We report a case of a splenic inflammatory pseudotumor (myofibroblastic tumor) in a 43-year-old man with a 5-year history of chronic bronchitis and sleep apnea syndrome. The patient was hospitalized because of a screen-detected splenic mass lesion. His sputum cultures revealed <I>Mycobacterium avium</I> complexes on only one occasion. Imaging studies revealed a 7 cm solitary tumorous lesion, and differential diagnoses of splenic hamartoma, hemangioma, lymphoma, and angiosarcoma were obtained from the radiologist. A splenectomy followed by pathological investigations was performed. By histology, the lesion contained fibroblastic or myofibroblastic spindle cell proliferations, accompanied by variable degrees of inflammatory cell infiltration. Ziehl-Neelsen staining did not reveal acid-fast bacteria. Immunohistochemically, the fibroblastic or myofibroblastic spindle cells were positive for vimentin, human smooth muscle actin, and muscle actin, but negative for desmin, CD8, CD21, CD23, CD35, p80, Epstein-Barr virus LMP, and human herpesvirus type 8. The infiltrating lymphoid cells demonstrated a nonneoplastic pattern. The results of <I>in situ</I> hybridization for Epstein-Barr virus encoded RNA were negative. The postoperative course was uneventful and he has had no recurrence in 22 months. His sleep apnea syndrome and chronic bronchitis have resolved spontaneously since the splenectomy. [<I>J Clin Exp Hematopathol 47(2) </I><I>: 83</I>-<I>88, 2007</I>]

DOI： 10.3960/jslrt.47.83

PubMed

CiNii Books

researchmap

その他リンク： http://search.jamas.or.jp/link/ui/2008186010

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：SPRINGER  

A number of the lysosomal storage diseases that have now been characterized are associated with intra-lysosomal accumulation of lipids, caused by defective lysosomal enzymes. We have previously reported neuronal accumulation of both alpha- and beta-synucleins in brain tissue of a GM2 gangliosidosis mouse model. Although alpha-synuclein has been implicated in several neurodegenerative disorders including Parkinson's disease, dementia with Lewy bodies and multiple system atrophy, its functions remain largely unclear. In our present study, we have examined a cohort of human lipidosis cases, including Sandhoff disease, Tay-Sachs disease, metachromatic leukodystrophy, beta-galactosialidosis and adrenoleukodystrophy, for the expression of alpha- and beta-synucleins and the associated lipid storage levels. The accumulation of alpha-synuclein was found in brain tissue in not only cases of lysosomal storage diseases, but also in instances of adrenoleukodystrophy, which is a peroxisomal disease. alpha-synuclein was detected in both neurons and glial cells of patients with these two disorders, although its distribution was found to be disease-dependent. In addition, alpha-synuclein-positive neurons were also found to be NeuN-positive, whereas NeuN-negative neurons did not show any accumulation of this protein. By comparison, the accumulation of beta-synuclein was detectable only in the pons of Sandhoff disease cases. This differential accumulation of alpha- and beta-synucleins in human lipidoses may be related to functional differences between these two proteins. In addition, the accumulation of alpha-synuclein may also be a condition that is common to lysosomal storage diseases and adrenoleukodystrophies that show an enhanced expression of this protein upon the elevation of stored lipids.

DOI： 10.1007/s00401-007-0264-z

Web of Science

PubMed

researchmap

記述言語：英語   出版者・発行元：LIPPINCOTT WILLIAMS & WILKINS  

DOI： 10.1097/RLU.0b013e31806541a4

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：SPRINGER TOKYO  

Follow-up colonoscopy of a 25-year-old Japanese man with ulcerative colitis (UC) who had undergone endoscopic mucosal resection twice for early colon cancers revealed the presence of a new 1.5-cm-diameter tumor in the sigmoid colon. It was diagnosed by preoperative biopsy as a poorly differentiated adenocarcinoma. Sigmoidectomy was performed, and the pathological findings revealed lymphoepithelioma-like carcinoma (LEC). In situ hybridization to detect Epstein-Barr virus (EBV)-encoded small RNAs showed positive signals in stromal lymphocytes, but weak signals in the tumor cells. The association between EBV and LEC was obscure in this case. Unlike typical UC-mediated colon cancers, the lesion was poorly differentiated, and negative for p53 signals immunohistochemically. These findings may hint at a novel mechanism of carcinogenesis in UC-mediated colorectal cancer.

DOI： 10.1007/s00535-006-1981-0

Web of Science

PubMed

researchmap

出版者・発行元：株式会社医学書院  

DOI： 10.11477/mf.1543101222

CiNii Research

researchmap

記述言語：日本語   掲載種別：研究論文（学術雑誌）  

In August 2000, a 62-year-old woman presented to another municipal hospital with macroscopic hematuria. Transurethral resection of bladder tumor (TUR-Bt) was performed. The pathological diagnosis was transitional cell carcinoma (TCC), G2 &gt
squamous cell carcinoma (SCC). TUR-Bt repeated in July 2003 indicated recurrence. The pathological diagnosis was TCC, G2. She was referred to our hospital in August 2003 because she desired bladder preservation. After cystoscopy and random biopsy, pathological diagnosis was TCC with squamous differentiation, G1-G2, pTis. She received 7 weekly intravesical bacillus Calmette-Guerin (BCG) instillations. In April 2004, TUR-Bt was repeated and multiple recurrences were found. The pathological diagnosis was TCC with squamous differentiation, G1-G2, pTa. She received 10 weekly intravesical Pirarubicin hydrochroride instillations. In August cystoscopy and random biopsy were performed for evaluation of the intavesical instillation treatment. Pathological diagnosis was atypical squamous cells. In November, cystoscopy revealed recurrence of a bladder tumor. After admission, a small papillary tumor and multiple flat lesion biopsies demonstrated SCC without obvious invasion. The patient underwent cystectomy. There were widespread areas of full thickness squamous atypia. Most of the bladder did not show appearance of typical TCC, but the final pathological diagnosis was TCC because the case developed from TCC and could not be diagnosed as pure SCC. The diagnosis of SCC in situ of bladder is difficult, and this may contribute to its rarity.

Scopus

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：PHARMACEUTICAL SOC JAPAN  

Sandhoff disease (SD) is an autosomal recessive GM2 gangliosidosis caused by the defect of lysosomal beta-hexosaminidase (Hex) beta-subunit gene associated with neurosomatic manifestations. Therapeutic effects of Hex subunit gene transduction have been examined on Sandhoff disease model mice (SD mice) produced by the allelic disruption of Hexb gene encoding the murine beta-subunit. We demonstrate here that elimination of GM2 ganglioside (GM2) accumulated in the fibroblastic cell line derived from SD mice (FSD) did not occur when the HEXB gene only was transfected. In contrast, a significant increase in the HexB (beta beta homodimer) activity toward neutral substrates, including GA2 (asialo-GM2) and oligosaccharides carrying the terminal N-acetylglucosamine residues at their non-reducing ends (G1cNAc-oligosaccharides) was observed. Immunoblotting with anti-human HexA (a,6 heterodimer) serum after native polyacrylamide gel electrophoresis (Native-PAGE) revealed that the human HEXB gene product could hardly form the chimeric HexA through associating with the murine a-subunit. However, co-introduction of the HEXA encoding the human a-subunit and HEXB genes caused significant corrective effect on the GM2 degradation by producing the human HexA. These results indicate that the recombinant human HexA could interspeciesly associate with the murine GM2 activator protein to degrade GM2 accumulated in the FSD cells. Thus, therapeutic effects of the recombinant human HexA isozyme but not human HEXB gene product could be evaluated by using the SD mice.

DOI： 10.1248/bpb.29.1564

Web of Science

PubMed

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：ELSEVIER SCI LTD  

Myxofibrosarcoma is common in the extremities, but rare in the head and neck region. Hypopharyngeal myxofibrosarcoma has not been reported previously. We report the first case of a patient with myxofibrosarcoma of the hypopharynx. We examined this patient once a month after the operation, and there has been no local recurrence and no distant metastasis. Sarcomas are rare in the hypopharynx, but we have to bear in mind their possibility. Though a low-grade myxofibrosarcoma is a low-grade malignancy, complete resection should be done. We have to pay more attention planning the treatment for neoplastic diseases. (C) 2005 Elsevier Ireland Ltd. All rights reserved.

DOI： 10.1016/j.anl.2005.07.004

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

A 55-year-old man, diagnosed with systemic sclerosis (SSc) for 20 years, was admitted to our hospital for exertional dyspnea and pleural effusion. Computed tomography scan and cytological findings of the pleural fluid suggested malignant mesothelioma. In the postmortem examination, the tumor was pathologically diagnosed as pseudomesotheliomatous adenocarcinoma (PMA) of the lung, classified into pleomorphic carcinoma with adenocarcinoma component according to the new World Health Organization guidelines. This is the first case report of SSc with PMA. © Japan College of Rheumatology 2006.

DOI： 10.1007/s10165-006-0472-8

Scopus

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：ELSEVIER GMBH, URBAN & FISCHER VERLAG  

A major challenge for the management of prostate cancer (PCa) patients is to predict the clinical course of the disease after radical prostatectomy. A previous comprehensive immunohistochemical analysis using an automated image analyzer suggested that prolyl isomerase Pin1 (hence Pin1) may be a potent predictor of recurrence in PCa patients. However, a detailed pathological standard for evaluating the Pin1 immunohistochemistry in PCa has not been established yet. We here introduce a practical scoring system for Pin1 immunostaining in PCa. Using this method, the immunoreactivity of tumor cell cytoplasm and nucleus was evaluated separately and then scored for four grades (Grade = 0-3). We defined the Pin1 score as the sum of both nuclear and cytoplasmic grades (Score = 0-6), and the cases were then divided into either a low Pin1 score group (&lt;= 2) or a high Pin1 score group (&gt;= 3). We examined the correlation between this scoring system and postoperative PSA recurrence for 78 PCa patients. PCa patients assigned to the high Pin1 score group demonstrated PSA relapse more frequently than those assigned to the low Pin1 score group (p &lt; 0.0001). This suggests that, at the common laboratory level, our Pin1 scoring system could be a useful tool for predicting the prognosis of PCa patients after surgery. (c) 2006 Elsevier GmbH. All rights reserved.

DOI： 10.1016/j.prp.2005.12.007

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：SPRINGER TOKYO  

We have established spontaneously immortalized Schwann cell lines from dorsal root ganglia and peripheral nerves of Sandhoff mice. One of the cell lines exhibited genetically and biochemically distinct features of Sandhoff Schwann cells. The enzyme activities toward 4-methylumbelliferyl N-acetyl-beta-D-glucosamine (beta-hexosaminidases A, B, and S) and 4-methylumbelliferyl N-acetyl-beta-D- glucosamine-6-sulfate (beta-hexosaminidases A and S) were decreased, and GM2 ganglioside accumulated in lysosomes of the cells. Incorporation of recombinant human beta-hexosaminidase isozymes expressed in Chinese hamster ovary cells into the cultured Sandhoff Schwann cells via cation-independent mannose 6-phosphate receptors was found, and the incorporated beta-hexosaminidase A degraded the accumulated GM2 ganglioside. The established Sandhoff Schwann cell line is useful for investigation and development of therapies for Sandhoff disease.

DOI： 10.1007/s10038-005-0278-0

Web of Science

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：ASSOC RESEARCH VISION OPHTHALMOLOGY INC  

PURPOSE. To investigate the effects of lysosomal storage on the morphologic appearance and the neurite outgrowth capability of the retina in a mouse model of G(M2) gangliosidosis ( Sandhoff disease).
METHODS. Histopathologic appearances of retinas in Sandhoff (SD) mice at 3 and 4 months of age were examined by light and electron microscopy. Retinas of SD mice and wild-type (WT) mice at 1, 2, and 4 months of age were cultured in collagen gel in the presence or absence of brain-derived neurotrophic factor ( BDNF), and neurite outgrowth was examined.
RESULTS. Morphologic studies revealed accumulation of G(M2) ganglioside in the retinal ganglion cells of SD mice in a time-dependent manner. The number of neurites from the retinal explants after 7 and 10 days in culture were significantly lower in 2- and 4-month-old SD mice than in the age-matched WT mice. The application of BDNF significantly improved neurite outgrowth from the retina in both SD and WT mice at 2 months of age. At 4 months of age, BDNF was much less effective at stimulating neurite outgrowth in the retina of SD mice than in retina of WT mice.
CONCLUSIONS. These results indicate that lysosomal storage of G(M2) ganglioside impairs the capability of neurite outgrowth in retinal ganglion cells in culture and that BDNF is effective at diminishing this impairment during the early stage of the disease.

DOI： 10.1167/iovs.05-0038

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Nephrin, a product of the NPHS1 gene, is a component of the slit diaphragms that are found between glomerular foot processes and is a crucial element for glomerular filtration barrier. Recently, nephrin has been focused in a number of studies of proteinuria development including various types of acquired glomerular diseases including minimal change nephrotic syndrome and membranous nephropathy. However, the precise role of nephrin in such acquired glomerular diseases is still unknown. To analyse the role of nephrin further, two kinds of anti-nephrin antibodies were raised in the rabbits and applied to an experimental mouse model of chronic graft-versus-host disease, in which (C57BL/10 × DBA/2) F1 mice developed clinically apparent severe proteinuria with significant glomerular lesions 7 weeks after parental DBA/2 cell transfer. Antibody-sandwich ELISA detected anti-nephrin antibodies during week 2 to week 6, with the peak at week 2 or week 4. Colocalization of nephrin and IgG on week 4, week 6, and week 8 was revealed by confocal microscopic analysis, suggesting that in situ immune complex formation with nephrin in glomerular lesion. Taken together, it seems to be suggested nephrin and its autoantibody have a certain role in the development of glomerular lesion in our model mice. © 2005 British Society for Immunology.

DOI： 10.1111/j.1365-2249.2005.02838.x

Scopus

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：BLACKWELL PUBLISHING ASIA  

The authors have recently reported the presence of characteristic foamy swelling/degeneration (giant lamellar body degeneration, GLBD) of type II pneumocytes in the lungs affected by Hermansky-Pudlak syndrome (HPS)-associated interstitial pneumonia (HPSIP), and proposed the hypothesis that GLBD may be the triggering factor in the development of HPSIP (Virchows Arch 2000; 437: 304-13). The purpose of the present paper was to investigate the lung pathology of pale ear (ep) mouse, a mouse model of HPS1, and of beige (bg) mouse, a mouse model of Chediak-Higashi syndrome (CHS) with a reference to GLBD and associated pathologic changes. GLBD was found in both ep and bg mice soon after birth, and increased in severity as the mice grew older. Younger mice had only GLBD with no evidence of interstitial change. Aged bg mice showed the most prominent GLBD and patchy areas of alveolar collapse accompanied by lymphocytic infiltration and slight fibrosis. Aged ep mice with less severe GLBD than bg mice of comparable ages also had a slight tendency to develop interstitial inflammation but no fibrosis. The pneumocytes with GLBD were immunoreactive for surfactant protein B and composed of giant lamellar bodies ultrastructurally, findings which were almost identical to those of human GLBD. The results of the present study support the hypothesis that GLBD may play an important role in the development of HPSIP. Ep and bg mice, especially the latter, may be useful mouse models of HPSIP.

DOI： 10.1111/j.1440-1827.2005.01811.x

Web of Science

PubMed

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：BLACKWELL PUBLISHING LTD  

Sandhoff disease is a lysosomal storage disease caused by simultaneous deficiencies of beta-hexosaminidase A ( HexA; ab) and B ( HexB; betabeta), due to a primary defect of the beta-subunit gene (HEXB) associated with excessive accumulation of GM2 ganglioside ( GM2) and oligosaccharides with N-acetylhexosamine residues at their non-reducing termini, and with neurosomatic manifestations. To elucidate the neuroinflammatory mechanisms involved in its pathogenesis, we analyzed the expression of chemokines in Sandhoff disease model mice (SD mice) produced by disruption of the murine Hex beta-subunit gene allele (Hexb-/-). We demonstrated that chemokine macrophage inflammatory protein-1 alpha (MIP-1alpha) was induced in brain regions, including the cerebral cortex, brain stem and cerebellum, of SD mice from an early stage of the pathogenesis but not in other systemic organs. On the other hand, little changes in other chemokine mRNAs, including those of RANTES (regulated upon activation, normal T expressed and secreted), MCP-1 ( monocyte chemotactic protein-1), SLC (secondary lymphoid-tissue chemokine), fractalkine and SDF-1 (stromal derived factor-1), were detected. Significant up-regulation of MIP-1alpha mRNA and protein in the above-mentioned brain regions was observed in parallel with the accumulation of natural substrates of HexA and HexB. Immunohistochemical analysis revealed that MIP-1alpha-immunoreactivity (IR) in the above-mentioned brain regions of SD mice was co-localized in Iba1-IR-positive microglial cells and partly in glial fibrillary acidic protein (GFAP)-IR-positive astrocytes, in which marked accumulation of N-acetylglucosaminyl (GlcNAc)-oligosaccharides was observed from the presymptomatic stage of the disease. In contrast, little MIP1alpha-IR was observed in neurons in which GM2 accumulated predominantly. These results suggest that specific induction of MIP-1alpha might coincide with the accumulation of GlcNAc-oligosaccharides due to a HexB deficiency in resident microglia and astrocytes in the brains of SD mice causing their activation and acceleration of the progressive neurodegeneration in SD mice.

DOI： 10.1111/j.1471-4159.2005.02986.x

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）  

We have established spontaneously immortalized Schwann cell lines from dorsal root ganglia and peripheral nerves of Sandhoff mice. One of the cell lines exhibited genetically and biochemically distinct features of Sandhoff Schwann cells. The enzyme activities toward 4-methylumbelliferyl N-acetyl-beta-D-glucosamine (beta-hexosaminidases A, B, and S) and 4-methylumbelliferyl N-acetyl-beta-D-glucosamine-6-sulfate (beta-hexosaminidases A and S) were decreased, and GM2 ganglioside accumulated in lysosomes of the cells. Incorporation of recombinant human beta-hexosaminidase isozymes expressed in Chinese hamster ovary cells into the cultured Sandhoff Schwann cells via cation-independent mannose 6-phosphate receptors was found, and the incorporated beta-hexosaminidase A degraded the accumulated GM2 ganglioside. The established Sandhoff Schwann cell line is useful for investigation and development of therapies for Sandhoff disease.

DOI： 10.1007/s10038-005-0278-0

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：SAGE PUBLICATIONS INC  

Eight cases of reactive nodular fibrous pseudotumor of the gastrointestinal tract are presented. The patients included 6 males and 2 females between the ages of 1 and 68 years (mean age 41.5 years). Three tumors involved the small intestine, and 5 of the investigated lesions were located in the large bowel. Of these, 2 originated in the sigmoid colon, 1 in the cecum, 1 in the appendix, and 1 in the large bowel not otherwise specified. The tumors&apos; size varied from 3 to 10 cm in the greatest diameter (mean 6.2 cm). Histologically they were composed of stellate or spindle shaped cells resembling fibroblasts arranged haphazardly or in intersecting fascicles, embedded in a collagen-rich stroma, with sparse intralesional mononuclear cells frequently arranged in lymphoid aggregates. Immunohistochemically, the lesions were positive for vimentin (7/7), smooth muscle actin (8/8), muscle-specific actin (5/7), cytokeratins AE1/AE3 (6/7), and CAM 5.2 (1/7), and antigen CD68 (1/7). No case (0/8) reacted positively with antibody to CD117 (c-kit). Genetically no substitutions, deletions, or insertions occurred in exon 11 in all analyzed samples. Likewise, no deletions or insertions in part of exon 9 were observed. Ultrastructurally the tumor cells revealed features typical of myofibroblasts. According to the morphologic, immunohistochemical, and ultrastructural features mentioned above, especially to the positivity of low-molecular-weight cytokeratins, we propose this lesion to be related to a proliferation of multipotential subserosal cells rather than ordinary myofibroblasts or fibroblasts.

DOI： 10.1177/106689690401200409

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：LIPPINCOTT WILLIAMS & WILKINS  

Adult ABO-incompatible liver transplantation has been known to be associated with markedly desperate outcomes. Antibody-mediated rejection (AMR) has been recognized as one of the primary causes of these desperate outcomes, but its clinical features and significance have not been well understood. Recently, some clinicians have succeeded in improving the outcome of adult ABO-incompatible liver transplantation. However, in some transplant patients undergoing these treatments, AMR has still led to graft losses. We recently encountered two patients suffering from AMR after adult ABO-incompatible liver transplantation and remedied their conditions with various therapeutic modalities including direct hepatic infusion therapy and gamma-globulin bolus infusion therapy combined with plasmapheresis. In this article, we describe the clinical features of these patients and the therapeutic strategies we applied. Furthermore, we show the histologic course of the recovery from AMR in the second patient, from whom we were able to extract serial liver biopsies.

DOI： 10.1097/01.TP.0000137264.99113.2B

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：W B SAUNDERS CO  

Recent studies have indicated that aberrant beta-catenin expression may be a common denominator for the morular formation of tumors from various anatomic sites. The evidence for the influence of female sex hormones in the formation of morules has been circumstantial, most previous studies having failed to demonstrate female sex hormone receptors in the mondar cells. We investigated a possible role of estrogen receptor (ER)-beta in the occurrence of tumors that form morules with biotin-rich optically clear nuclei (BROCN)(BROCN-family tumors) and its possible relationship with aberrant nuclear/cytoplasmic (N/C) beta-catenin expression. We immunostained 14 BROCN-family tumors, including 6 low-grade adenocarcinomas of the fetal lung type, 3 papillary thyroid carcinomas of cribriform-morular variant (CMV), 2 ovarian endometrioid tumors, 2 colonic adenocarcinomas, and 1 gallbladder adenoma, as well as 4 cases of endometrial tissue with BROCN during gestation, for ERbeta, ERalpha, progesterone receptor (PgR), beta-catenin, and, on a subset of cases, c-Fos and MIB-1 as well. BROCN in all 18 cases expressed ERbeta but not ERalpha or PgR. In the BROCN-family tumors, the morular cells and budding glandular cells expressed ERbeta in the cytoplasm and BROCN, which overlapped with the N/C expression pattern of beta-catenin. beta-catenin showed only membranous expression in the endometrial glands during gestation. In CMV and ovarian endometrioid tumors, nuclear expression of ERalpha and PgR were observed in association with N/C beta-catenin expression only in the glandular component. C-Fos was also constantly and strongly expressed in BROCN in all cases examined. The MIB-1 labeling index was low in the morular area, ranging from 1% to 3 %. The present study indicates that N/C co-localization of ERbeta and beta-catenin is a feature common to the morules with BROCN that appear in the BROCN-family tumors from various anatomic sites. Whether the estrogen-signaling pathway and the Wnt-signaling pathway have crosstalk, cooperating in the development of the BROCN-family tumors, awaits further study. (C) 2004 Elsevier Inc. All rights reserved.

DOI： 10.1016/j.humpath.2004.03.018

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：AMER SOC CLINICAL INVESTIGATION INC  

Mice containing a disruption of the Hexb gene have provided a useful model system for the study of the human lysosomal storage disorder known as Sandhoff disease (SD). Hexb(-/-) mice rapidly develop a progressive neurologic disease of ganglioside GM2 and GA2 storage. Our study revealed that the disease states in this model are associated with the appearance of antiganglioside autoantibodies. Both elevation of serum antiganglioside autoantibodies and IgG deposition to CNS neurons were found in the advanced stages of the disease in Hexb(-/-) mice; serum transfer from these mice showed IgG binding to neurons. To determine the role of these autoantibodies, the Fc receptor gamma gene (FcRgamma) was additionally disrupted in Hexb(-/-) mice, as it plays a key role in immune complex-mediated autoimmune diseases. Clinical symptoms were improved and life spans were extended in the Hexb(-/-)FcRgamma(-/-) mice; the number of apoptotic cells was also decreased. The level of ganglioside accumulation, however, did not change. IgG deposition was also confirmed in the brain of an autopsied SD patient. Taken together, these findings suggest that the production of autoantibodies plays an important role in the pathogenesis of neuropathy in SD and therefore provides a target for novel therapies.

DOI： 10.1172/JCI200419639

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：LIPPINCOTT WILLIAMS & WILKINS  

Sandhoff disease (SD) is a heritable lysosomal storage disease resulting from impaired degradation of GM2 ganglioside. The hallmark pathology of the SD model mouse brain is GM2 ganglioside accumulation in neurons. In the present study, we immunohistochemically investigated the neuronal pathology in SD mouse brains, and demonstrated neuronal accumulation of alpha- and beta-synucleins in addition to GM2 ganglioside. Synuclein-positive neurons were extensively observed throughout SD mouse brains, although the distribution of Beta-synuclein was less extensive than that of alpha-synuclein. Synuclein-positive neurons were negative to ubiquitin and PHF-tau. These findings suggest that neuronal synucleins may accumulate secondarily to GM2 ganglioside in SD mouse brains, and that neuronal accumulation of synucleins may be more critical than that of GM2 ganglioside for SD mice.

DOI： 10.1097/01.wnr.0000061017.47393.dc

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：SPRINGER-VERLAG  

Sandhoff disease is a severe neurodegenerative disorder with visceral involvement caused by mutations in the HEXB gene coding for the P subunit of the lysosomal hexosaminidases A and B. HEXB mutations result in the accumulation of undegraded substrates such as GM2 and GA2 in lysosomes. We evaluated the efficacy of cationic liposome-mediated plasmid gene therapy using the Sandhoff disease mouse, an animal model of a human lysosomal storage disease. The mice received a single intravenous injection of two plasmids, encoding the human alpha and beta subunits of hexosaminidase cDNAs. As a result, 10-35% of normal levels of hexosaminidase expression, theoretically therapeutic levels, were achieved in most visceral organs, but not in the brain, 3 days after injection with decreased levels by day 7. Histochemical staining confirmed widespread enzyme activity in visceral organs. Both GA2 and GM2 were reduced by almost 10% and 50%, respectively, on day 3, and by 60% and 70% on day 7 compared with untreated age-matched Sandhoff disease mice. Consistent with the biochemical results, a reduction in GM2 was observed in liver cells histologically as well. These initial findings support further development of the plasmid gene therapy against lysosomal diseases with visceral pathology.

DOI： 10.1007/s00109-002-0410-y

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：LIPPINCOTT WILLIAMS & WILKINS  

The salient histopathologic features of low-grade adenocarcinoma. of the fetal lung type (L-FLAC)/well-differentiated fetal adenocarcinoma (WDFA) include complex glandular structures and morules with biotin-rich optically clear nuclei. Interestingly, these characteristic features are shared by the cribriform-morular variant of papillary thyroid carcinoma, whose morphology is identical to that of familial adenomatous polyposis (FAP)-associated thyroid carcinoma. Furthermore, the single reported case of lung cancer associated with FAP was L-FLAC/WDFA. These observations lead us to hypothesize that up-regulation of the Writ signaling pathway underlies the development of L-FLAC/WDFA. To verify this hypothesis, 11 cases of L-FLAC/WDFA, including the one FAP-associated case, eight cases of high-grade adenocarcinoma. of the fetal lung type (H-FIAC), 24 cases of conventional pulmonary adenocarcinoma (CAC), and 13 fetal lungs were immunostained for beta-catenin. All cases of L-FLAC/WDFA showed predominantly aberrant nuclear/cytoplasmic expression, especially in budding glands and morules, whereas six of eight cases (75%) of H-FLAC and all but one case (96%) of CAC showed predominantly membranous expression. Fetal lungs showed nuclear/cytoplasmic expression restricted to the distal branching airway epithelium. Mutational analysis of exon 3 of the beta-catenin gene in five sporadic cases of L-FLAC/WDFA showed a point mutation at codon 34 and codon 37 in two cases, respectively. The present study indicates that up-regulating disturbances in the Wnt signaling pathway, including mutation of the beta-catenin gene, underlie tumorigenesis of L-FLAC/WDFA. The expression pattern of beta-catenin in L-FLAC/WDFA resembles that of the developing fetal lung airway. With the expression pattern of beta-catenin as a marker, most cases of H-FLAC as well as CAC appear to have different oncogenic pathways from cases of L-FLAC/WDFA. The present study together with other available data also suggests that abnormal up-regulation of the Writ signaling pathway may be a common denominator for the development of tumors with morular formation from a variety of anatomic sites.

DOI： 10.1038/modpathol.3880575

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：BLACKWELL PUBLISHING LTD  

Sandhoff disease is a heritable lysosomal storage disease resulting from impaired degradation of G(M2) ganglioside and related substrates. A mouse model of Sandhoff disease created by gene targeting displays progressive neurological manifestations, similar to patients with the disease. In the present in vivo and in vitro studies, we examined morphological and functional abnormalities of dorsal root ganglion (DRG) neurones in Sandhoff disease mice at an asymptomatic stage (approximately 1 month of age). Light microscopic studies with Nissl staining and immunocytochemistry suggested extensive intracytoplasmic storage of G(M2) ganglioside in the Sandhoff mouse DRG neurones. These findings were consistent with the results of electron microscopy, in which a huge number of pleomorphic inclusion bodies immunoreactive for G(M2) ganglioside were present in the cytoplasm of the neurones. The inclusion bodies were also identified in satellite cells and Schwann cells in the Sandhoff mouse DRG. The survival ratios of DRG neurones after 1, 2, 4 and 6 days in culture were significantly lower in the Sandhoff mice than in the age-matched heterozygous mice. The ratio of neurite-bearing cells on poly-L-lysine-coated dishes after 2 days in culture was also lower by approximately 10% in the Sandhoff mice compared to the heterozygotes, but additional coating of laminin onto poly-L-lysine dramatically enhanced the neurite extension from the neurones in both groups of mice. These results indicate that accumulation of G(M2) ganglioside in DRG neurones impairs the capability of the neurones to survive in vitro, although viable neurones from the Sandhoff mice in culture can regenerate neurites nearly as well as unaffected neurones.

DOI： 10.1046/j.1365-2990.2002.00366.x

Web of Science

Scopus

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：SPRINGER-VERLAG  

Beta-hexosaminidases are important enzymes for lipid and saccharide metabolism in the brain. In mice deficient in these enzymes, indigestible metabolic intermediates deposit in neurons. Inclusions such as membranous cytoplasmic bodies (MCB) and zebra bodies were seen in neurons of Tay-Sachs (TS) model mice, Sandhoff's disease (SD) model mice, and double knockout (DKO) mice. However, the cerebral perivascular macrophages discovered by Mato are active in the uptake of waste products and regarded as scavenger cells under steady-state conditions. We observed that indigestible components derived from neurons were taken up by the perivascular macrophages of TS mice by pinocytosis, but those of SD and DKO mice contained only pale inclusions and had marked vacuolations, and pinocytosis was rarely observed. Histochemically, the inclusions in the perivascular macrophages of TS mice were positive for the PAS stain, but those of SD and DKO mice were negative. In addition, the perivascular cells of TS mice expressed clear positive immunoreactivity against BM-8 and F4/80, but those of DKO mice had very weak BM-8 and F4/80 immunoreactivity. These differences between TS, SD, and DKO mice are based on their metabolism of oligosaccharides and glycosaminoglycans (GAG). Thus, hexosaminidase B is more important for keeping normal morphology and function of perivascular macrophages than hexosaminidase A. The foamy cells that appeared along the cerebral microvessels in lipidosis and saccharidosis were identified as perivascular macrophages (Mato's fluorescent granular perithelial cells: FGP cells).

DOI： 10.1007/s004010100441

Web of Science

PubMed

researchmap

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：SPRINGER-VERLAG  

Although usual interstitial pneumonia (UIP)-like IP has been known as the most serious complication of Hermansky-Pudlak syndrome (HPS), its pathologic features and pathogenesis are poorly understood. We investigated biopsied and autopsied lung tissues from five patients who died of UIP-like IP associated with HPS (HPSIP). The salient histopathologic features of HPSIP observed were: (1) alveolar septa displaying florid proliferation of type-2 pneumocytes (2PCs) with characteristic foamy swelling/degeneration; (2) patchy fibrosis with lymphocytic and histiocytic infiltration centered around respiratory bronchioles, occasionally showing constrictive bronchiolitis; and (3) honeycomb change without predilection for the lower lobes or subpleural area. Those peculiar 2PCs were histochemically characterized by the over accumulation of phospholipid, immunohistochemically by a weak positivity for surfactant protein, and ultrastructurally by the presence of numerous giant lamellar bodies that compressed the nucleus with occasional cytoplasmic disruption, together suggesting a form of cellular degeneration with an over accumulation of surfactant (giant lamellar body degeneration). The present study strongly indicates that there is a basic defect in the formation/secretion process of surfactant by the 2PCs in HPS, which may well be the triggering factor for the HPSIP development. Other factors, such as macrophage dysfunction, may be working synergistically for further acceleration of the inflammatory process.

Web of Science

researchmap

J-GLOBAL

researchmap

J-GLOBAL

researchmap

J-GLOBAL

researchmap

J-GLOBAL

researchmap

J-GLOBAL

researchmap

J-GLOBAL

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

60代男性。主訴は口腔内違和感。右口腔底に硬結を触れる腫瘍認めた為、経口腔的腫瘍摘出術を施行。口腔粘膜直下の境界明瞭な白色腫瘍で、組織学的に淡明で広い胞体を有する異型細胞が敷石状・胞巣状にみられ、一部に粘液も認めた。粘表皮癌明細胞型との鑑別を要したが、二色解離プローブを用いたfluorescence in situ hybridization(FISH)法でEWSR1およびATF1遺伝子に再構成を認め、明細胞癌の診断に至った。補助診断マーカーとしてFISH法が有用であった。(著者抄録)

J-GLOBAL

researchmap

記述言語：日本語   出版者・発行元：神奈川県医師会  

researchmap

記述言語：日本語  

J-GLOBAL

researchmap

記述言語：日本語   出版者・発行元：日本脳腫瘍病理学会  

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：日本語   出版者・発行元：日本脳腫瘍病理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語  

J-GLOBAL

researchmap

記述言語：日本語   出版者・発行元：日本外科系連合学会  

症例は54歳女性.既往として神経線維腫症I型:von Recklinghausen病(以下vRD)と診断されていた.血便を主訴に他院を受診し,上下部内視鏡が施行されたが異常なく,当院紹介となった.カプセル内視鏡検査で,上部小腸に小腸腫瘍を疑わせる所見を認めた.経口および経肛門小腸鏡が施行され,gastrointestinal stromal tumor(以下GIST)と診断された.腹腔鏡補助下での小腸部分切除術および腫瘍切除術を施行した.腫瘍は空腸から回腸までに存在し,2mmから50mmまでで,計12個を確認した.いずれもが小腸GISTであった.vRDには近年GIST合併例の報告が散見される.今回われわれは稀なvRDに合併した多発生小腸GISTを腹腔鏡補助下に切除を行った1例を経験した.この症例のような多発する特徴のある病変に対し,腹腔鏡による観察は有用であると考え報告する.(著者抄録)

researchmap

その他リンク： https://search-tp.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2019&ichushi_jid=J01066&link_issn=&doc_id=20190308090009&doc_link_id=10.4030%2Fjjcs.44.49&url=https%3A%2F%2Fdoi.org%2F10.4030%2Fjjcs.44.49&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_3.gif

記述言語：日本語   出版者・発行元：神奈川県医師会  

J-GLOBAL

researchmap

J-GLOBAL

researchmap

J-GLOBAL

researchmap

J-GLOBAL

researchmap

J-GLOBAL

researchmap

記述言語：日本語  

J-GLOBAL

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：日本語  

J-GLOBAL

researchmap

記述言語：日本語  

J-GLOBAL

researchmap

記述言語：日本語   出版者・発行元：神奈川県医師会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本皮膚悪性腫瘍学会  

76歳、男性。初診5年前より右眼に流涙を認め、他院眼科で生検され右眼窩部導管癌と診断された。切除希望なく放射線療法を施行された。照射1年後、右頸部に暗赤色斑が出現、徐々に拡大し、嚥下困難も出現し、近医より当科紹介受診した。初診時、右耳前部から頸部にかけて広範囲に境界明瞭な硬結を伴う暗赤色斑を認め血管肉腫を疑った。PET/CT画像で右頸部リンパ節転移、多発骨転移、肺転移を認めた。生検病理組織より右眼窩部導管癌の皮膚転移と診断した。病変部への放射線治療を施行、カルボプラチン、エピルビシンによる化学療法を3回施行し、皮膚症状は著明に改善、嚥下困難も軽快した。その後、左頸部への新たな皮膚転移、右口腔内有棘細胞癌を認め、緩和的放射線療法を施行した。眼窩部の導管癌は報告が少なく皮膚転移は稀である。丹毒様紅斑を呈した機序について、腫瘍細胞がリンパ管内で塞栓を形成し、真皮・皮下組織への浸潤を来したためと推察した。(著者抄録)

researchmap

記述言語：日本語  

J-GLOBAL

researchmap

記述言語：日本語  

J-GLOBAL

researchmap

記述言語：日本語  

J-GLOBAL

researchmap

記述言語：英語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

J-GLOBAL

researchmap

J-GLOBAL

researchmap

J-GLOBAL

researchmap

記述言語：日本語   出版者・発行元：(株)星和書店  

嚥下困難、発汗、微熱を主症状に悪性症候群を発症し、dantrolene sodiumの投与などにより発熱や筋強剛は改善するも、嚥下障害や呼吸不全が遷延、その後回復期に至って突然死した統合失調症の60代女性の剖検例を報告した。剖検の結果、気管内に胃内容物と思われる半液状の物質を認めるとともに、肺のうっ血の所見も認め、死因としては逆流した胃内容物の誤嚥が疑われた。悪性症候群の症状群においてdantrolene sodiumが有効であるのは、発熱、筋強剛などの一部症状に対してだけであり、嚥下や呼吸に関してはかなりの長期にわたって注意深くモニターすることが必要であると考えられた。(著者抄録)

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本肝臓学会  

症例は48歳の男性。C型慢性肝炎に対し肝庇護療法を行い経過観察中に急激に腹水貯留、下腿浮腫、多発するリンパ節腫脹が出現し紹介受診となった。リンパ節生検の結果から多中心性キャッスルマン病と診断した。ステロイド投与により症状改善を認め、低アルブミン血症や血小板減少も改善を認めた。腹水の減少後に施行した肝生検で新犬山分類A1F2相当であり肝線維化の急激な進行は否定的であった。ステロイドは投与量を漸減し治療開始から約2年7ヵ月で投与を終了した。キャッスルマン病に対する治療終了から約2年後に、C型慢性肝炎に対して経口抗ウイルス薬(Direct Acting Antivirals:DAAs)の投与を行い、ウイルス学的著効を得た。C型慢性肝炎の経過中に急激に腹水貯留、浮腫、リンパ節腫脹を認めた場合にはキャッスルマン病等のリンパ増殖性疾患も鑑別に挙げる必要性を強く認識させられた啓発的な症例であった。(著者抄録)

DOI： 10.2957/kanzo.58.494

CiNii Books

researchmap

その他リンク： http://search.jamas.or.jp/link/ui/2017404159

記述言語：日本語   出版者・発行元：神奈川県臨床細胞学会  

researchmap

記述言語：日本語   出版者・発行元：医学図書出版(株)  

researchmap

記述言語：英語   出版者・発行元：日本脳腫瘍病理学会  

researchmap

記述言語：日本語  

J-GLOBAL

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：日本語   出版者・発行元：医学図書出版(株)  

症例1は66歳男性で、経尿道的膀胱腫瘍切除術(TUR-BT)を施行した。膀胱注入療法を計6回施行した。膀胱鏡で膀胱内全体に黄白色の物質が付着し、膀胱粘膜の観察が困難となった。Mycobacterium bovis感染疑いの診断で、イソニアジド+リファンピシン+エタンブトール(INH+RFP+EB)を2ヵ月間、INH+RFPを4ヵ月間投与した。内服1ヵ月後で尿培養は陰性化した。膀胱癌の再発は認めていない。症例2は69歳男性で、TUR-BTを施行した。BCG膀注療法を6回施行し、維持療法を行ったが、頻尿と排尿時痛にて2回で終了した。MRIで前立腺右葉に前立腺癌疑いの所見があり、前立腺針生検+膀胱鏡を施行した。BCG膀注療法に伴う類上皮細胞肉芽腫と診断した。抗結核薬は使用せず対症療法で経過観察中である。膀胱癌の再発は認めていない。症例3は68歳男性で、TUR-BTを施行した。BCG膀注療法を6回、維持療法を2回施行した。排尿時痛が悪化し、MRIはBCG膀注療法後肉芽腫性膀胱炎+前立腺炎疑いの所見であった。現在対症療法で経過観察中である。膀胱癌の再発は認めていない。

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：日本語   出版者・発行元：(公社)日本整形外科学会  

J-GLOBAL

researchmap

J-GLOBAL

researchmap

記述言語：日本語  

J-GLOBAL

researchmap

記述言語：日本語   出版者・発行元：東日本整形災害外科学会  

J-GLOBAL

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

Sclerosing Perivascular Epithelioid Cell Tumor(Sclerosing PEComa)の頻度は低く、診断に苦慮するか、他の組織型と診断されている可能性がある。我々は同腫瘍の1例を経験した。症例は50歳、女性。腫瘍は17mm大で、腎から外向性に突出してみられた。組織学的には紡錘形あるいは上皮様腫瘍細胞が索状や島状に増殖し、太い索状の硝子様間質が介在した。小血管を取り囲むように上皮様腫瘍細胞が増殖する像もみられた。免疫組織化学的に複数の筋系マーカーがびまん性に陽性を示す一方、メラノサイト系マーカーはHMB45のみが少数の細胞に陽性を示した。平滑筋系腫瘍と見誤らないよう注意が必要であると思われた。(著者抄録)

researchmap

記述言語：日本語  

J-GLOBAL

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

Web of Science

researchmap

記述言語：日本語   出版者・発行元：神奈川県医師会  

researchmap

記述言語：日本語   出版者・発行元：神奈川県医師会  

researchmap

記述言語：日本語   出版者・発行元：(公社)日本婦人科腫瘍学会  

researchmap

記述言語：日本語   出版者・発行元：横浜市立大学医学会  

CiNii Books

researchmap

その他リンク： http://search.jamas.or.jp/link/ui/2015275887

記述言語：日本語   出版者・発行元：(一社)日本泌尿器科学会総会事務局  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：日本臨床外科学会  

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：日本語   出版者・発行元：一般社団法人日本外科学会  

CiNii Books

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：医学図書出版(株)  

膵癌の予後改善のために有効な治療は、R0手術の達成である。Borderline resectableもしくはlocally advancedの膵頭部癌の診療において、術前抗癌剤放射線療法(NACRT)導入により、根治治療の可能性が高まってきている。閉塞性黄疸、胆管炎で発症した膵頭部癌のNACRT期間を安定、確実に遂行するため、partial cover metaric stent(PCMS)の早期導入が肝要である。MDCT診断に立脚したNCCNガイドライン2012をもとに、診断を行い、およそ3ヵ月の治療期間を中断なく行うことで、R0達成率を高められる可能性がある。さらにPCMSはcost面からも、今後の術前内視鏡的内瘻術の、第一選択となる可能性がある。(著者抄録)

researchmap

記述言語：日本語   出版者・発行元：日本脳腫瘍病理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本腎臓学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：一般社団法人日本外科学会  

CiNii Books

researchmap

記述言語：日本語   出版者・発行元：神奈川県医師会  

researchmap

記述言語：日本語   出版者・発行元：(公社)日本婦人科腫瘍学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語  

J-GLOBAL

researchmap

記述言語：日本語   出版者・発行元：(一社)日本消化器内視鏡学会-関東支部  

researchmap

記述言語：日本語   出版者・発行元：日本婦人科病理学会  

researchmap

記述言語：英語   出版者・発行元：GEORG THIEME VERLAG KG  

DOI： 10.1055/s-0030-1256782

Web of Science

researchmap

記述言語：日本語   出版者・発行元：(一社)日本乳癌学会  

researchmap

記述言語：日本語   出版者・発行元：(公社)日本臨床細胞学会  

researchmap

記述言語：日本語  

J-GLOBAL

researchmap

記述言語：日本語  

CiNii Books

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：(一財)日本消化器病学会  

researchmap

記述言語：日本語   出版者・発行元：神奈川県臨床細胞学会  

背景:子宮体部扁平上皮癌はまれな疾患である。今回われわれは子宮内膜細胞診が子宮体部原発扁平上皮癌の診断に有用であった1例を経験したので報告する。症例:68歳、女性。不正性器出血を自覚し近医を受診。その後当院へ紹介受診となった。子宮内膜細胞診では、オレンジG好性あるいはライトグリーン濃染性で、核に粗大顆粒状のクロマチンを有する扁平上皮癌を疑わせる細胞が多数認められた。内膜組織診においても多数の扁平上皮癌胞巣が認められ、子宮体部原発の扁平上皮癌が疑われた。子宮頸部円錐切除術も施行されたが扁平上皮癌は認められなかった。子宮体癌I期の診断で手術を施行し、子宮体部扁平上皮癌Ib期の診断を得た。摘出組織では扁平上皮癌細胞が本来の子宮内膜面に認められ、さらに筋層浸潤をきたしていた。結論:子宮体部原発扁平上皮癌はまれな腫瘍であるが、その存存を念頭におき、内膜細胞診、組織診および画像所見を総合し術前診断を行う必要があると考えられた。(著者抄録)

researchmap

記述言語：日本語  

CiNii Books

researchmap

記述言語：日本語   出版者・発行元：(一社)日本リンパ網内系学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：(一社)日本病理学会  

researchmap

記述言語：日本語   出版者・発行元：がんの子供を守る会  

4例の小児卵巣悪性胚細胞腫瘍に対し,carboplatin,etoposide,bleomycinによるJEB療法を施行した.初発時の年齢は9歳から14歳,腫瘍の病理組織診断はyolk sac tumorが2例,dysgermiminomaがl例,mixed germ cell tumorが1例であった.初発時から45～61か月現在,全例が無病生存中である.卵巣悪性胚細胞腫瘍に対して行われてきたBEP(bleomycin,etoposide,cisplatin)療法に比しJEB療法は肺毒性や聴器毒性,腎毒性が少なく,安全に施行し得た.晩期障害の点においてJEB療法は小児に有用な治療法であると考えられた.

CiNii Books

researchmap

その他リンク： https://projects.repo.nii.ac.jp/?action=repository_uri&item_id=263299

記述言語：日本語   出版者・発行元：(一社)日本消化器内視鏡学会-関東支部  

症例は63歳、男性。膵腫大、膵管狭細像に加え、Vater乳頭部腫大と同部位でのIgG4陽性形質細胞浸潤を認め、自己免疫性膵炎と診断された。これらの所見は、いずれもステロイド治療開始後より改善を認めた。自己免疫性膵炎における診断、治療効果の判定の補助にVater乳頭部所見と、同部位へのIgG4陽性形質細胞浸潤の程度が有用であることが示唆された。(著者抄録)

DOI： 10.11641/pde.75.2_126

researchmap

その他リンク： http://search.jamas.or.jp/link/ui/2010068114