記述言語：英語   掲載種別：研究論文（学術雑誌）  

Gastrointestinal duplications are congenital malformations that are usually observed in pediatric patients. Diagnosis in adulthood is quite rare, and preoperative diagnosis of gastrointestinal duplication is difficult, particularly in the small intestine. We encountered an extremely rare adult case of duplication of the jejunum, which showed a stomach-like form diagnosed using double-balloon enteroscopy (DBE). The patient was an 18-year-old male who had been experiencing upper abdominal pain and vomiting repeatedly without any triggers for 3 years. Various examinations were performed, but no cause of symptoms was found. DBE revealed a narrow opening of the lumen at the upper jejunum, and the lumen was covered with mucosal folds similar to those of the stomach. Enteroclysis via DBE showed a tubular structure on the mesenteric side of the jejunum. We diagnosed a jejunal tubular duplication with ectopic gastric mucosa and underwent partial small bowel resection. The patient's abdominal symptoms resolved. From this, DBE can be a useful tool for diagnosing intestinal duplication in adults. We believe that this case and literature review will facilitate the accurate and prompt diagnosis of small intestinal duplication.

DOI： 10.1007/s12328-022-01596-7

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Neuroendocrine neoplasms of the colon and rectum are colorectal epithelial neoplasms with neuroendocrine differentiation. A platinum regimen used for small cell lung cancer is the currently recommended chemotherapy for gastroenteropancreatic neuroendocrine carcinomas (GEP-NECs), regardless of the organ. The BRAF V600E mutation has been recently reported as a druggable driver mutation in colorectal NECs. In BRAF V600E mutant colorectal cancer, a combination of BRAF inhibitor and anti-epidermal growth factor receptor (EGFR) antibody, with or without a MEK inhibitor, is recommended. Here, we report the case of 77-year-old man who had lymph node recurrence after surgery for primary ascending colonic NEC. Two cytotoxic regimens, cisplatin plus irinotecan and modified FOLFOX6, were administered as first- and second-line chemotherapies with no remarkable response observed. At this point, genetic analysis confirmed the tumor harbored a BRAF V600E mutation. Thus, a regimen of BRAF inhibitor plus anti-EGFR antibody was administered. After commencing this regimen, carcinoembryonic antigen levels decreased within normal range, and there was dramatic shrinkage of the lymph node metastases observed by chest and abdominal computed tomography scans. To our knowledge, this is the first reported case of a colorectal NEC responding to a BRAF inhibitor and anti-EGFR antibody.

DOI： 10.1007/s12328-022-01599-4

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記述言語：英語  

DOI： 10.1002/pbc.29501

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記述言語：日本語   掲載種別：研究論文（学術雑誌）  

A 72-year-old man had a chief complaint of anal pain and difficulty in defecation. He was diagnosed with adenocarcinoma by biopsy from a tumor of the anal canal. A computed tomography scan revealed neither regional lymph node metastasis nor distant metastasis. Hence, he was diagnosed with cT3N0M0, cStage Ⅱa anal canal cancer. Preoperative capecitabine- based chemoradiotherapy(CRT)(50.4 Gy in 28 fractions of 1.8 Gy each)was implemented. Digital rectal examination and imaging evaluation 8 weeks after preoperative CRT revealed that the tumor had shrunk. Fifteen weeks after preoperative CRT, laparoscopic abdominoperineal resection was performed. The pathological findings showed mucinous adenocarcinoma associated with anal fistula. At present, 12 months after the operation, no local recurrence and distant metastasis has been detected under follow-up evaluations.

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Synovial sarcoma is a rare malignant soft-tissue tumor that is prevalent in adolescents and young adults, and poor prognosis has been reported in patients with metastatic lesions. Chimeric antigen receptor (CAR) T-cell therapy is an emerging novel therapy for solid tumors; however, its application in synovial sarcoma has not yet been explored. METHODS: A novel human epidermal growth factor receptor 2 (HER2)-targeted CAR containing scFv-FRP5, CD8α hinge and transmembrane domains as well as 4-1BB costimulatory and CD3ζ signaling domains was developed. Three synovial sarcoma cell lines that expressed the fusion transcript SS18-SSX1/2/4 were used in the study. Cytokine secretion assay, cytotoxicity assay, and real-time cell analysis experiments were conducted to confirm the function of T cells transduced with the CAR gene. RESULTS: High cell-surface expression of HER2 was observed in all the cell lines. HER2-targeted/4-1BB-costimulated CAR T cells specifically recognized the synovial sarcoma cells, secreted interferon gamma and tumor necrosis factor alpha, and exerted cytotoxic effects in these cells. CONCLUSION: To the best of our knowledge, this is the first study to indicate that HER2-targeted CAR T cells are directly effective against molecularly defined synovial sarcoma cells. Furthermore, our findings might set the basis for developing improved CAR T cell-based therapies for chemo-refractory or relapsed synovial sarcoma.

DOI： 10.1016/j.tranon.2021.101227

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記述言語：日本語   掲載種別：研究論文（学術雑誌）  

A 70-year-old female with liver metastases from gastrointestinal stromal tumor(GIST)that were found 3 months after partial gastrectomy for the primary GIST underwent Auchincloss operation for left breast cancer with ipsilateral axillary lymph node metastases. The diagnosis was microinvasive ductal cancer that was pT1miN1M0, pStage ⅡA, hormone receptor negative, and HER2 positive. Given the impact of this cancer on the prognosis of liver metastases of GIST, imatinib therapy, but not adjuvant chemotherapy, was started promptly for breast cancer after surgery. Four months after the surgery, left subclavian lymph node recurrence of breast cancer was found. Since the liver metastases of GIST had been stable, imatinib was discontinued, and paclitaxel and anti-HER2 therapy were administered. After confirming tolerability, imatinib was carefully added in combination. Because the lymph nodes shrank and liver metastases of GIST were stable, both anti-HER2 therapy and imatinib were continued. There are few reports of combined chemotherapy for synchronous double cancer, and we report our experience in which careful treatment was required.

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Hepatocyte nuclear factor-4α (HNF4α) presents in multiple isoforms generated using alternative promoter (P1 and P2) and splicing. Neither conservation of tissue distribution of HNF4α isoforms, nor presence of alternative promoter usage is known. In this study, to detect the expression of HNF4α in some species of animals, we have applied monoclonal antibodies against P1 (K9218) and P2 (H6939) promoter-driven and P1/P2 promoter-driven H1415 HNF4α for immunohistochemistry and western blot analysis. Antibody K9218 was observed in the hepatocytes, proximal tubules of the kidney, and epithelial cells in the mucosa of the small intestine and colon of rats, chicken, and tortoise, whereas antibody H6939 signal were detected in the stomach, pancreas, bile duct, and pancreatic duct of human and rats. The signal for antibody K9218 was recognized in tissues of a wide range of mammals, bird, reptile, amphibian, and fish as well. Antibody H1415 displayed a positive reaction in hepatocytes and intestinal epithelial cells in chicken and tortoise, whereas the bile duct, mucosal epithelial cells in the stomach, or pancreas in these animals were negative. Western blotting showed the binding of the antibody with HNF4α protein from each animal. The sequence of human HNF4α was 100% identical to murine and rat HNF4α, 88.9% to chicken, 77.8% to Xenopus HNF4α, and 81.5% to medaka. However, the specific part of human and invertebrate Drosophila HNF4 shares only 14.8% sequence identity. This antibody is useful for detecting HNF4α isoforms in a wide range of vertebrates, and suggests many insights into animal evolution.

DOI： 10.1002/jemt.23848

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記述言語：英語  

BACKGROUND: Colorectal mucinous adenocarcinoma is a rare subtype of colorectal cancer and is characterized by an abundance of mucin in the tumor. In addition, the colorectal mucinous adenocarcinoma often demonstrates poor differentiation in the histology of tumor cells and poor prognosis compared with those with adenocarcinoma. Here, we present the case of a young woman with colonic mucinous adenocarcinoma showing significantly rapid progression within four months of immunosuppressant therapy for Henoch-Schönlein purpura. CASE SUMMARY: Here we report a rare case of ascending colon mucinous adenocarcinoma with lymph node and liver metastases which developed and progressed rapidly within four months during the treatment of Henoch-Schönlein purpura using corticosteroids. The systemic screening examinations showed no tumors before the immunosuppressant therapy. Fortunately, the patient was successfully treated with chemotherapy. CONCLUSION: While no direct evidence that the immunosuppressants accelerated the tumor development, the case presenta tion and review of the literature demonstrated that surveillance for malignancies before and during treatment with immunosuppressive agents is essential.

DOI： 10.12998/wjcc.v9.i30.9182

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記述言語：英語  

Spinal dissemination in epithelioid glioblastoma can be diagnosed by cerebrospinal fluid cytology and liquid biopsy to detect BRAF V600E mutation.

DOI： 10.1002/ccr3.4551

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

We herein report a rare case of HCC metastases to the ovary and peritoneum in a 61-year-old female patient who has achieved 11-year survival with multidisciplinary therapy. The patient was diagnosed with HCC during balloon angioplasty performed for Budd-Chiari syndrome in 1994 and underwent partial hepatectomy twice. Five years after the second hepatectomy, allochronic recurrence of a single nodule detected in S8 was treated by radiofrequency ablation, followed by percutaneous ethanol injection therapy and stereotactic body radiotherapy. However, her α-fetoprotein level rose to 1862 ng/mL within one year and computed tomography revealed a large pelvic tumor suggesting HCC metastasis to the ovary. The subsequent laparotomy revealed one 11-cm left ovarian tumor, one small right ovarian nodule, and numerous peritoneal nodules. Bilateral salpingo-oophorectomy and peritoneal resection of as many nodules as possible were performed. Combination therapy with intravenous 5-fluorouracil plus cisplatin and ramucirumab monotherapy effectively suppressed tumor progression with maintenance of hepatic functional reserve, and she has achieved long-term survival of 11 years, illustrating that multidisciplinary therapy with favorable hepatic functional reserve maintenance can contribute to long-term survival in HCC with extrahepatic spread.

DOI： 10.1007/s12328-021-01434-2

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記述言語：英語  

DOI： 10.1016/j.jos.2018.09.009

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

The characteristics of the tumor immune microenvironment remains unclear in liposarcomas, and here we aimed to determine the prognostic impact of the tumor immune microenvironment across separate liposarcomas subtypes. A total of 70 liposarcoma patients with three subtypes: myxoid liposarcoma (n = 45), dedifferentiated liposarcoma (n = 17), and pleomorphic liposarcoma (n = 8) were enrolled. The presence of tumor infiltrating lymphocytes (CD4+ , CD8+ , FOXP3+ lymphocytes) and CD163+ macrophages and expression of HLA class I and PD-L1 were assessed by immunohistochemistry in the diagnostic samples; overall survival and progression-free survival were estimated from outcome data. For infiltrating lymphocytes and macrophages, dedifferentiated liposarcoma and pleomorphic liposarcoma patients had a significantly higher number than myxoid liposarcoma patients. While myxoid liposarcoma patients with a high number of macrophages were associated with worse overall and progression-free survival, dedifferentiated liposarcoma patients with high macrophage numbers showed a trend toward favorable prognosis. Expression of HLA class I was negative in 35 of 45 (77.8%) myxoid liposarcoma tumors, whereas all dedifferentiated liposarcoma and pleomorphic liposarcoma tumors expressed HLA class I. The subset of myxoid liposarcoma patients with high HLA class I expression had significantly poor overall and progression-free survival, while dedifferentiated liposarcoma patients with high HLA class I expression tended to have favorable outcomes. Only four of 17 (23.5%) dedifferentiated liposarcomas, two of eight (25%) pleomorphic liposarcomas, and no myxoid liposarcoma tumors expressed PD-L1. Our results demonstrate the unique immune microenvironment of myxoid liposarcomas compared to other subtypes of liposarcomas, suggesting that the approach for immunotherapy in liposarcomas should be based on subtype.

DOI： 10.1007/s00262-021-02928-1

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記述言語：日本語   出版者・発行元：(一社)日本病理学会  

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Jaundice may be persistent in drug-induced liver injury associated with vanishing bile duct syndrome. However, recurrent jaundice is atypical, following bile flow restoration. Here, we report a 28-year-old man with prolonged, recurrent jaundice (more than 300 days) and combined immunodeficiency (CID) of B-cells, T-cells, and natural killer (NK) cells. Hypogammaglobulinemia was observed throughout his hospitalization, and peripheral blood flow cytometry detected a few B-cells (2% of CD19 + cells and 2% of CD20 + cells). We further detected the dysfunction of T-cells and NK cells. Based on these findings, CID was diagnosed. We presumed that hypogammaglobulinemia was related to the jaundice. After regular injections of intravenous immunoglobulin (IVIG), the stool color gradually turned brown. However, the color returned to white as IgG levels decreased. The brown-to-white stool pattern was repeated with another IVIG administration, suggesting that the patient's serum immunoglobulin levels were related to the jaundice. On follow-up, IVIG was performed every two to three weeks, and his total bilirubin improved gradually. Immunoglobulin replacement therapy could be one of the treatment choices for jaundice with CID.

DOI： 10.1007/s12328-021-01347-0

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Early detection of head and neck carcinoma (HNC) as superficial HNC (SHNC) identified using recently developed optical techniques, such as magnifying endoscopy and narrow-band imaging (NBI), in combination with endoscopic surgeries enables minimally invasive treatment with favorable outcomes for HNC. This study aimed to identify the predictive factors for the rare but important clinical issue of SHNC, namely cervical lymph node metastasis (CLNM), following endoscopic resection. METHODS: Sixty-nine patients with SHNC who underwent endoscopic resection were enrolled in the study. Clinical data, preoperative endoscopic findings, pathological findings, and treatment outcomes were retrospectively reviewed. Because the pharyngeal mucosa lacks the muscularis mucosa, we measured tumor thickness in permanent pathology as an alternative to the depth of invasion. Correlations with the occurrence of CLNM were statistically examined. RESULTS: The 5-year disease-specific survival rate was 100%. Of 69 patients, 3 (4.3%) developed CLNM. All had subepithelial but not epithelial tumors. The 0-IIa type in the macroscopic findings, type B2/B3 vessels in narrow-band imaging, tumors ≥ pathological stage T2, lymphatic invasion, positive surgical margins, and tumor thickness >1,000 μm showed significant correlations with CLNM following endoscopic resection. Furthermore, the classification of type B vessels was significantly associated with tumor thickness. CONCLUSION: The treatment outcomes following endoscopic resection for SHNC were favorable. The risk of CLNM following endoscopic resection in SHNC can be predicted by several preoperative endoscopic and postoperative pathological findings. Among them, the classification of type B vessels, which correlated with both tumor thickness and CLNM, might be a useful predictive factor.

DOI： 10.3389/fsurg.2021.813260

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記述言語：日本語   掲載種別：研究論文（学術雑誌）  

A 48-year-old female discovered a mass in her left axilla. A thorough examination resulted in a diagnosis of left invasive lobular carcinoma(ILC)of the accessory mammary gland with wide ductal spread. Considering the wide ductal spread, massive resection of the left axilla mass, left lymph node dissection, and a latissimus dorsi musculocutaneous flap procedure were performed. However, histological analysis revealed ILC measuring 80×50 mm with lymph node metastases(5/23)and extensive cancer spread, resulting in a positive surgical margin. It is important to recognize the characteristics of ILC, axillary accessory breast cancer, and the axilla in a treatment strategy.

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記述言語：日本語   掲載種別：研究論文（学術雑誌）  

A 73-year-old man presented with anemia, and gastroscopy showed a nonpigmented tumor in the esophagogastric junction. The result of the tumor biopsy initially suspected poorly differentiated adenocarcinoma. However, additional immunohistochemical examination revealed malignant melanoma. The final diagnosis was amelanotic malignant melanoma of the esophagogastric junction with adrenal and spinal metastasis. Although immunotherapy was performed, the patient died 132 days after diagnosis.

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記述言語：日本語   出版者・発行元：(株)癌と化学療法社  

症例は73歳、男性。貧血精査目的の上部消化管内視鏡検査で、食道胃接合部に白色から発赤調で易出血性の不整な隆起性腫瘤を認めた。生検では充実型低分化腺癌が疑われたが確定診断が得られず、追加で施行した免疫組織化学染色で悪性黒色腫と診断した。食道胃接合部原発の悪性黒色腫、cT3N1M1(副腎、骨)、cStage IVb(食道癌取扱い規約第15版)に対して、ペムブロリズマブの投与を行ったが肺転移が出現し原病死した。本例は腫瘍の主局在が胃側にあり、メラニン欠乏性悪性黒色腫であったため典型的な肉眼的・組織学的所見が得られず診断に難渋した。食道胃接合部の腫瘍においても、メラニン欠乏性悪性黒色腫の可能性を念頭に置いて診断を行う必要がある。(著者抄録)

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その他リンク： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2020&ichushi_jid=J00296&link_issn=&doc_id=20210118600115&doc_link_id=%2Fab8gtkrc%2F2020%2F004713%2F115%2F2083b2085%26dl%3D3&url=http%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fab8gtkrc%2F2020%2F004713%2F115%2F2083b2085%26dl%3D3&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_4.gif

記述言語：英語   掲載種別：研究論文（学術雑誌）  

Ribosomal RNA (rRNA), the most abundant non-coding RNA species, is a major component of the ribosome. Impaired ribosome biogenesis causes the dysfunction of protein synthesis and diseases called "ribosomopathies," including genetic disorders with cancer risk. However, the potential role of rRNA gene (rDNA) alterations in cancer is unknown. We investigated germline and somatic single-nucleotide variants (SNVs) in the rDNA promoter region (positions -248 to +100, relative to the transcription start site) in 82 lung adenocarcinomas (LUAC). Twenty-nine tumors (35.4%) carried germline SNVs, and eight tumors (9.8%) harbored somatic SNVs. Interestingly, the presence of germline SNVs between positions +1 and +100 (n = 12; 14.6%) was associated with significantly shorter recurrence-free survival (RFS) and overall survival (OS) by univariate analysis (p < 0.05, respectively), and was an independent prognostic factor for RFS and OS by multivariate analysis. LUAC cell line PC9, carrying rDNA promoter SNV at position +49, showed significantly higher ribosome biogenesis than H1650 cells without SNV. Upon nucleolar stress induced by actinomycin D, PC9 retained significantly higher ribosome biogenesis than H1650. These results highlight the possible functional role of SNVs at specific sites of the rDNA promoter region in ribosome biogenesis, the progression of LUAC, and their potential prognostic value.

DOI： 10.3390/cells9112409

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

A 75-year-old woman with liver cirrhosis was admitted for treatment of portal vein thrombosis (PVT). Computed tomography (CT) showed PVT, massive ascites, and multiple abdominal organ embolism. Blood tests revealed a decreased liver function (Child-Pugh grade C). Language impairment followed by progressive left hemi-paralysis was subsequently detected. Magnetic resonance imaging revealed multiple small acute cerebral infarctions and, on CT, a 30-mm bladder tumour; a biopsy specimen examination showed squamous cell carcinoma. Her general condition worsened rapidly, and the best supportive care was chosen. Our findings suggest that, in patients with PVT, Trousseau syndrome should be considered, even in cases of liver cirrhosis.

DOI： 10.2169/internalmedicine.4112-19

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

An asymptomatic epiphrenic diverticulum (ED) was diagnosed in a man undergoing annual esophagogastroduodenoscopy (EGD) at another hospital 40 years before he presented to our hospital at age 63 years for his annual EGD. However, because substantial food retention was found in the ED, we could not confirm a lesion. After the retained food was removed endoscopically, a second EGD showed a reddish, flat lesion with an elevated mass within the ED. Endoscopic ultrasonography indicated that the elevated mass was deep in the submucosal layer. An esophagram showed that the ED was approximately 80 mm in diameter, which is considered large. An endoscopic biopsy of the lesion confirmed squamous cell carcinoma. Total esophagectomy was performed. Microscopic examination revealed well-differentiated to moderately differentiated squamous cell carcinoma invading the adventitia at the elevated lesion. The final pathological stage was pT3N0M0. There was no evidence of recurrence for 3 years during the quarterly follow-up examinations. To our knowledge, this case involved the longest asymptomatic term (40 years) since the ED was detected. A review of 18 reported cases of carcinoma in an ED indicated that advanced cancer has a poor prognosis. Periodic follow-up of ED patients is essential for early diagnosis.

DOI： 10.1007/s12328-020-01098-4

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1016/j.jtcvs.2019.04.015

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

A histological grading system of chromophobe renal cell carcinoma (chRCC) is highly desirable to identify approximately 5-10% of tumors at risk for progression. Validation studies failed to demonstrate a correlation between the four-tiered WHO/ISUP grade and outcome. Previous proposals with three-tiered chromophobe grading systems could not be validated. In this study, the presence of sarcomatoid differentiation, necrosis, and mitosis was analyzed in a Swiss cohort (n = 42), an Italian cohort (n = 103), a German cohort (n = 54), a Japanese cohort (n = 119), and The Cancer Genome Atlas cohort (n = 64). All 3 histological parameters were significantly associated with shorter time to tumor progression and overall survival in univariate analysis. Interobserver variability for identification of these parameters was measured by Krippendorff's alpha coefficient and showed high concordance for the identification of sarcomatoid differentiation and tumor necrosis, but only low to medium concordance for the identification of mitosis. Therefore, we tested a two-tiered tumor grading system (low versus high grade) based only on the presence of sarcomatoid differentiation and/or necrosis finding in the combined cohorts (n = 382). pT stage, patient's age (> 65 vs ≤ 65), lymph node and/or distant metastasis, and the two-tiered grading system (low versus high grade) were significantly associated with overall survival and were independent prognostic parameters in multivariate analysis (Cox proportional hazard). This multi-institutional evaluation of prognostic parameters suggests tumor necrosis and sarcomatoid differentiation as reproducible components of a two-tiered chromophobe tumor grading system.

DOI： 10.1007/s00428-019-02710-w

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

The legends of Figs. 1 and 3 in the published original version of the above article are incorrect.

DOI： 10.1007/s00428-020-02786-9

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記述言語：英語  

DOI： 10.1016/j.lungcan.2019.11.012

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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 differentiate between low- and high-risk types of thymoma using quantitative 3D shape analysis of CT images. MATERIALS AND METHODS. This retrospective study included 44 patients with a pathologic diagnosis of thymoma. Two radiologists semiautomatically contoured CT images of the tumors and evaluated 3D shape parameters-namely, quantitative indicators of surface smoothness, including sphericity, ellipsoidality, and discrete compactness. The visual CT findings that were analyzed included longest diameter, shape (round-oval, lobulated, or irregular), calcification, cystic or necrotic changes, and enhancement pattern (homogeneous or heterogeneous). The difference and discriminating performance between low-risk (types A, AB, and B1) and high-risk (types B2 and B3) thymomas were statistically assessed. Interobserver agreement was determined using the concordance correlation coefficient. RESULTS. Twenty-three low-risk and 21 high-risk thymomas were identified on the basis of pathologic findings. The median values of sphericity and ellipsoidality were significantly higher for low-risk thymomas than for high-risk thymomas (for sphericity, 0.566 vs 0.517; for ellipsoidality, 0.941 vs 0.875; p < 0.05 for both). The AUC values of sphericity and ellipsoidality were 0.704 and 0.712, respectively. The best cutoff values were 0.528 and 0.919 for sphericity and ellipsoidality, respectively. Risk assessment combining these cutoff values and the mode of tumor detection (incidental detection or detection based on the presence of symptoms) improved the AUC value to 0.856 (sensitivity, 81.0% [17 of 21 patients]; specificity, 82.6% [19 of 23 patients]). All 3D shape parameters showed almost perfect interobserver agreement (concordance correlation coefficient, > 0.90). The visual CT findings were not significantly different between low- and high-risk thymomas (p > 0.05 for all). CONCLUSION. Quantitative 3D shape analysis has excellent reproducibility, and combining this technique with information on the detection mode helps differentiate low- from high-risk thymomas.

DOI： 10.2214/AJR.19.21844

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Chromophobe renal cell carcinoma (chRCC) is a renal tumor subtype with a good prognosis, characterized by multiple chromosomal copy number variations (CNV). The World Health Organization (WHO) chRCC classification guidelines define a classic and an eosinophilic variant. Large cells with reticular cytoplasm and prominent cell membranes (pale cells) are characteristic for classic chRCC. Classic and eosinophilic variants were defined in 42 Swiss chRCCs, 119 Japanese chRCCs and in whole-slide digital images of 66 chRCCs from the Cancer Genome Atlas (TCGA) kidney chromophobe (KICH) dataset. 32 of 42 (76.2%) Swiss chRCCs, 90 of 119 (75.6%) Japanese chRCCs and 53 of 66 (80.3%) TCGA-KICH were classic chRCCs. There was no survival difference between eosinophilic and classic chRCC in all three cohorts. To identify a genotype/phenotype correlation, we performed a genome-wide CNV analysis using Affymetrix OncoScan® CNV Assay (Affymetrix/Thermo Fisher Scientific, Waltham, MA, USA) in 33 Swiss chRCCs. TCGA-KICH subtypes were compared with TCGA CNV data. In the combined Swiss and TCGA-KICH cohorts, losses of chromosome 1, 2, 6, 10, 13, and 17 were significantly more frequent in classic chRCC (p < 0.05, each), suggesting that classic chRCC are characterized by higher chromosomal instability. This molecular difference justifies the definition of two chRCC variants. Absence of pale cells could be used as main histological criterion to define the eosinophilic variant of chRCC.

DOI： 10.3390/cancers11101492

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Bone and soft tissue tumors are derived from mesenchymal cells, and they are hard to treat. Receptor-activator of nuclear factor-kappa B ligand (RANKL) is an essential cytokine for osteoclast differentiation and activation and is expressed on the surface of osteoblasts or stromal cells. In this study, to explore the potential of denosumab treatment for soft tissue tumors, we analyzed the expression profiles of RANKL mRNA in 425 tumor specimens of 33 histological types by real-time RT-PCR. Denosumab is a monoclonal antibody that prevents the binding of RANKL to receptor-activator of nuclear factor-kappa B (RANK). For comparison, the relative expression levels of RANK and osteoprotegerin (OPG) mRNAs were also measured. OPG functions as a soluble decoy receptor for RANKL. Higher expression levels of RANKL mRNA were detected in calcifying aponeurotic fibroma, fibrosarcoma, calcifying epithelioma, myositis ossificans, heterotopic calcification, giant cell tumor of the tendon sheath (GCTTS), and pigmented villonodular synovitis (PVNS), compared with the levels of other tumor types. Moreover, the expression levels of RANK mRNA were highest in GCTTS, followed by myositis ossificans and PVNS, whereas the expression levels of OPG mRNA were greatly varied among these histological types. We then analyzed RANKL protein expression by immunohistochemistry in 57 tumor specimens with higher expression levels of RANKL mRNA. RANKL-positive cells were detected in GCTTS, PVNS, myositis ossificans, heterotopic calcification, and calcifying aponeurotic fibroma. In conclusion, RANKL is expressed in subsets of soft tissue tumors with calcification, and denosumab is a potential therapeutic option for soft tissue tumors expressing RANKL.

DOI： 10.1620/tjem.248.87

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記述言語：日本語   出版者・発行元：(株)癌と化学療法社  

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

We report a case of inferior vena cava (IVC) thrombosis caused by compression by a giant liver cyst. A 68-year-old man with a 1-day history of abdominal pain was referred to another hospital. Ultrasonography (US) and enhanced computed tomography (CT) showed a multilobular cyst on the right liver lobe that had increased to 300 mm in diameter from 90 mm 18 months earlier. Thrombosis was detected in the IVC, which was compressed by the cyst. Percutaneous transhepatic cyst drainage achieved no significant change in size. Cytological analysis from the percutaneous drainage tube fluid showed no evidence of malignancy. He was referred to our hospital for further assessment and treatment. Enhanced US using perfluorobutane, CT, and magnetic resonance imaging showed no tumorous lesions in the cyst. Thus, we diagnosed it as a multilobular cyst with no evidence of malignancy. A 3-week course of heparin resulted in the successful resolution of the thrombosis. Cystectomy was subsequently performed and pathological examination showed a multifocal cyst consisting of central suppurative inflammatory exudation and hemorrhagic material, with no malignancy. This case demonstrates that giant, expanding, non-tumorous cysts can cause IVC thrombosis. Careful treatment using heparin successfully resolved the thrombosis and allowed successful cystectomy.

DOI： 10.1007/s12328-018-0885-x

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Neurofibromatosis type 1 (NF1) is an autosomal dominant disease of the skin and soft tissue. Aneurysms associated with NF1 can occur, but a secondary aneurysm rupture is very rare, with very few cases reported in literature. CASE PRESENTATION: We describe the case of a 67-year-old female with NF1 who underwent endovascular aneurysm repair (EVAR) for an abdominal aortic aneurysm (AAA) rupture. She developed a type Ib endoleak requiring a redo-EVAR. Eighteen days after her primary operation, she was found to have two new left colic artery aneurysms. She required emergency surgery consisting of a left hemicolectomy and transverse colon colostomy. Pathology showed neurofibromatous changes to the peri-vasculature tissue, consistent with her underlying disease. CONCLUSIONS: Although rare, secondary aneurysms can occur following AAA repair. Patients with soft tissue connective tissue disorders, like NF1, may be at an increased risk for development of these secondary aneurysms. Endovascular repair appears to be a safe approach for NF1 patients with AAA, but endovascular management can be challenging in the setting of NF1. Surgeons should be ready to convert to open surgery if the patient displays persistent signs of bleeding or structural changes related to connective tissue disorders like NF1.

DOI： 10.1186/s40792-019-0570-4

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Objective: This study aimed to examine the natural course and relapse rate of IgG4-related ophthalmic disease (IgG4-ROD) after debulking surgery in Japanese patients. Methods and analysis: This retrospective review included patients with IgG4-ROD who did not undergo further treatment following debulking surgery. The patients were diagnosed between January 2009 and December 2018 at the Department of Ophthalmology and Pathology, Niigata University Medical and Dental Hospital. The main outcome measures included postoperative IgG4-ROD recurrence rate and differences between patients with and without recurrent disease. Results: Fifteen patients (six male, 9 female; 61.8±16.2 years) were included. Twelve patients (80.0%) had dacryoadenitis disease and three patients (20.0%) had orbital fat tissue disease. About 70%-100% of the lesion was resected in the debulking surgery and the pathological diagnosis was rendered. A definitive diagnosis was made in 13 cases (86.7%) and a probable diagnosis in 2 cases (13.3%). Patients were followed up for 39.0±25.5 months following operation. All patients had lesion volume reduction and patients with dacryoadenitis had eyelid swelling improvement after surgery. Two patients (13.3%) had disease recurrence and six patients (40.0%) had extraophthalmic lesions. There was no statistically significant difference in clinical features between relapsed and non-recurring cases. Conclusion: We observed a 13.3% relapse rate following debulking surgery in patients with IgG4-ROD who did not undergo further treatment. This rate is lower than the documented relapse rate of 30%-70% following oral prednisolone therapy. Therefore, debulking surgery may be a treatment option for IgG4-ROD.

DOI： 10.1136/bmjophth-2019-000295

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記述言語：日本語   出版者・発行元：日本消化器病学会-甲信越支部  

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

The association between the immune status within the tumor microenvironment and prognosis in synovial sarcoma is not well understood. We aimed to investigate the tumor immune microenvironment and analyze its prognostic impact for patients with synovial sarcoma. A total of 36 primary patients who were treated in our institution were retrospectively evaluated. Infiltration of lymphocytes (CD4+, CD8+, and FOXP3+), CD163+ macrophages, and expression of human leukocyte antigen (HLA) class I and programmed death ligand 1 (PD-L1) were evaluated by immunohistochemistry. Moreover, we investigated PD-L1 and programmed death ligand 2 (PD-L2) mRNA expression in 19 of the 36 cases, using real-time PCR. The Kaplan-Meier method was used to estimate overall survival and progression-free survival. Infiltration of lymphocytes and macrophages varied among the patients. Furthermore, the expression of HLA class I was negative or downregulated in 11 specimens. No PD-L1 expression was observed using immunohistochemistry. Moreover, although PD-L1 mRNA expression was observed in 18 of 19 specimens, the expression level was low. A higher infiltration of CD8+ or FOXP3+ lymphocytes in patients was associated with a favorable overall survival. In addition, a higher infiltration of CD163+ macrophages indicated a significantly worse overall and progression-free survival. Infiltration of CD4+ lymphocytes, HLA class I, PD-L1, and PD-L2 expression were not associated with patient prognosis. This represents the first report investigating the tumor immune microenvironment as a prognostic factor in synovial sarcoma, indicating that CD163+ macrophages are associated with tumor progression. Our results underscore the clinical significance of the tumor immune microenvironment in synovial sarcoma.

DOI： 10.1111/cas.13769

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記述言語：英語  

DOI： 10.1007/s00270-018-1958-4

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1038/ajg.2017.481

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記述言語：日本語   出版者・発行元：(公社)日本医学放射線学会  

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Bone leiomyosarcoma is a rare primary osseous malignant tumor with a high metastatic potential. Similar to other bone sarcomas, high histological grade and tumor stage are predictive of a poor outcome. We herein present our experience with treating a 64-year-old woman with bone leiomyosarcoma accompanied by multiple bone metastases. A biopsy revealed occasional osteoclast-like giant cells. In addition to radiation therapy, the osteoclastogenesis inhibitor denosumab was administered but the patient did not undergo adjuvant chemotherapy or surgery. Good clinical and short-term radiological responses to denosumab have been observed for 2 years. Therefore, denosumab may represent a viable treatment option without the need for adjuvant chemotherapy.

DOI： 10.3892/mco.2017.1489

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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  

We report a case of gastric-type intraductal papillary-mucinous neoplasm (IPMN) of the pancreas protruding into the main pancreatic duct with scant mucin secretion which was indistinguishable from intraductal tubulopapillary neoplasm (ITPN) preoperatively. A 71-year-old woman was referred to our hospital following acute pancreatitis for evaluation of an intraductal tumor of the pancreas arising in the main pancreatic duct (MPD). Although the tumor was shown as a filling defect in the MPD with a dilatation of the MPD in the body and tail of the pancreas on MRCP and ERCP, no secreted mucin was visible on ERCP. A Whipple pancreaticoduodenectomy with a regional lymph node dissection was performed with preoperative diagnosis of IPMN or ITPN. Gross inspection of the resected specimen revealed a 15-mm pedunculated tumor arising at the MPD without visible mucin. Histologic examination revealed that the tumor was composed of closely-packed tubular glands, similar to the pyloric gland of the stomach with minimal cytologic atypia and a small amount of mucin was found in the tumor gland microscopically. In the pancreatic duct adjacent to the tumor, small papillary projections were shown. After confirming the diagnosis of gastric-type IPMN with low-grade dysplasia, she was given no adjuvant chemotherapy and remains well with no evidence of the disease 48 months after resection.

DOI： 10.5833/jjgs.2017.0064

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記述言語：英語  

Duodenal gastrointestinal stromal tumors (GISTs) are extremely rare disease entities, and the extraluminal type is difficult to diagnose. These tumors have been misdiagnosed as pancreatic tumors; hence, pancreaticoduodenectomy has been performed, although partial duodenectomy can be performed if accurately diagnosed. Developing a diagnostic methodology including endoscopic ultrasonography (EUS) and fine-needle aspiration (FNA) has allowed us to diagnose the tumor directly through the duodenum. Here, we present a case of a 50-year-old woman with a 27-mm diameter tumor in the pancreatic uncus on computed tomography scan. EUS showed a well-defined hypoechoic mass in the pancreatic uncus that connected to the duodenal proper muscular layer and was followed by endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA). Histological examination showed spindle-shaped tumor cells positively stained for c-kit. Based on these findings, the tumor was finally diagnosed as a duodenal GIST of the extraluminal type, and the patient underwent successful mass resection with partial resection of the duodenum. This case suggests that EUS and EUS-FNA are effective for diagnosing the extraluminal type of duodenal GISTs, which is difficult to differentiate from pancreatic head tumor, and for performing the correct surgical procedure.

DOI： 10.4253/wjge.v9.i12.583

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory bone disorder that generally occurs in children and predominantly affects the long bones with marginal sclerosis. We herein report two cases of adult-onset CRMO involving the tibial diaphysis bilaterally, accompanied by polyarthritis. Magnetic resonance imaging (MRI) showed both tibial osteomyelitis and high intensity of the extensive lower leg muscles. Anti-interleukin-6 therapy with tocilizumab (TCZ) effectively controlled symptoms and inflammatory markers in both patients. High intensity of the lower leg muscles detected by MRI also improved. These cases demonstrate that CRMO should be included in the differential diagnosis of adult patients with bone pain, inflammation, and high intensity of the muscles detected by MRI. TCZ may therefore be an effective therapy for muscle inflammation of CRMO.

DOI： 10.2169/internalmedicine.8473-16

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Histiocytic sarcoma, a rare hematopoietic neoplasm with evidence of histiocytic differentiation, is often refractory to conventional chemotherapy and radiotherapy, and its prognosis is generally dismal. The optimal management of this malignancy has not been established. We report a case of 8-year-old girl with histiocytic sarcoma involving the left femur. The tumor rapidly responded to a combination of cladribine and high-dose cytosine arabinoside, an aggressive salvage regimen for refractory Langerhans cell histiocytosis, and became impalpable during the first cycle. The patient has remained in complete remission more than 7 years from diagnosis.

DOI： 10.1007/s12185-017-2202-8

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: A solitary fibrous tumour (SFT) is an unusual neoplasm typically found in soft tissues. Although SFTs can arise in the bones, they very rarely arise in the vertebral arch. Here, we describe a case of a SFT that arose in the vertebral arch of the first lumbar (L1) spinal vertebrae and mimicked osteosarcoma. CASE PRESENTATION: A 49-year-old woman presented with a 2-month history of lower back pain and a lumbar region mass. Magnetic resonance imaging demonstrated a heterogeneously enhanced mass in the L1 vertebral arch. The patient received neoadjuvant chemotherapy, followed by a surgical procedure comprising an anterior spinal fusion and en bloc resection. Histologically, our initial diagnosis was osteosarcoma. The postoperative course was uneventful, and the patient received adjuvant chemotherapy. However, the tumour metastasised to the lung 5 years after the first surgery, and a second surgery was performed for lung tumour resection. The histology of the metastatic lung tumour appeared similar to that of the malignant SFT, and the specimen from the first surgery was re-examined. Immunohistochemically, the tumour was positive for STAT6. Reverse transcription-polymerase chain reaction revealed a NAB2-STAT6 fusion gene, thus confirming our final diagnosis of malignant SFT. The patient died of disease progression 8 years after the first surgery; however, there was no evidence of local recurrence. CONCLUSIONS: Malignant SFT in the vertebral arch is extremely rare and very difficult to distinguish histologically an osteoid from lace-like collagen. STAT6 immunostaining is useful for distinguishing malignant SFTs from other neoplasms. Although it is difficult to completely resect a SFT arising from the spine, we demonstrated the feasibility of an en bloc resection of spinal tumours arising from posterior elements, without local recurrence.

DOI： 10.1186/s12957-017-1161-0

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記述言語：日本語   出版者・発行元：日本脳腫瘍病理学会  

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

The prognosis of alveolar soft part sarcoma is poor, despite the slow growth of the tumor. A number of cases with spontaneous regression of this rare tumor have been reported. Although the mechanisms underlying spontaneous regression remain uncertain, local immune reaction may be a possible contributing factor. Immunohistochemical expression of human leukocyte antigen (HLA) class I, cluster of differentiation (CD) 3, CD4, CD8, CD20, CD45, CD56, CD68, CD138 and CD163 were assessed in a series of 10 alveolar soft part sarcomas, and the expression profiles were associated with patients' clinicopathological parameters. Expression of HLA class I was observed in almost all the tumor cells of all cases. CD8(+) cells were identified in all tumors with varying densities. Moderate infiltration of CD8(+) cells was detected in three patients; one of these patients survived with long-term tumor remission. Infiltration of CD10(+), CD20(+), CD56(+) or CD138(+) cells was not revealed in all tumors. Moderate-diffuse infiltration of CD163(+) cells was observed in all tumors. To the best of our knowledge, the present study represents the first report of intratumoral immune cells in alveolar soft part sarcoma. Frequent expression of HLA class I in tumor cells was observed. CD8(+) cells were identified at various densities and CD163(+) cells were observed in alveolar soft part sarcoma. Moderate infiltration of CD8(+) cells in patients with a good prognosis may indicate the antitumor effects of immune cells in alveolar soft part sarcoma.

DOI： 10.3892/ol.2017.5696

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Meigs' syndrome is defined as the co-existence of benign ovarian fibroma or fibroma-like tumor, ascites, and pleural effusion. In contrast, pseudo-Meigs' syndrome is defined as the co-existence of other ovarian or pelvic tumors, ascites, and pleural effusion. In Meigs' and pseudo-Meigs' syndromes, ascites and pleural effusion resolve promptly after the complete resection of the ovarian or pelvic tumor(s). Secondary ovarian tumors from colorectal gastrointestinal metastases rarely cause pseudo-Meigs' syndrome; only 11 cases of pseudo-Meigs' syndrome secondary to colorectal cancers have been reported in the literature. Therefore, the prognosis and etiology of pseudo-Meigs' syndrome caused by ovarian metastasis from colorectal cancers remain unclear. CASE PRESENTATION: We report here a rare case of pseudo-Meigs' syndrome caused by ovarian metastases from sigmoid colon cancer with long-term survival. A 47-year-old woman presented with abdominal distention of 1-month duration. She developed acute dyspnea 2 weeks after the initial presentation. Colonoscopy and computed tomography revealed sigmoid colon cancer with an ovarian metastasis, along with massive ascites and bilateral pleural effusion. Emergency operation, including bilateral oophorectomy and sigmoidectomy, was performed. Subsequently, ascites and bilateral pleural effusion resolved rapidly. Curative hepatic resection was performed for liver metastases 29 months after the first operation, and as of this writing, the patient is alive with no evidence of a disease 78 months after the first operation. In general, colorectal cancer with ovarian metastasis is hard to cure, and long-term survival in patients with colorectal cancer with pseudo-Meigs' syndrome is rare. Our experience suggests that curative resection for pseudo-Meigs' syndrome caused by ovarian metastasis from colorectal cancer may offer long-term survival. CONCLUSIONS: Our experience suggests that pseudo-Meigs' syndrome can occur in a patient with colorectal cancer after metastasis to the ovaries, causing massive ascites and pleural effusion. Aggressive treatment, including R0 resection, for this disease if allowed by the patient's general condition may offer long-term survival.

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Myoepithelial carcinoma of the breast is an extremely rare tumor composed entirely of malignant spindle cells with myoepithelial differentiation. The majority of previously reported cases have mainly described the clinicopathological features of the disease, and few have presented cytogenetic data. We herein present the case of a 48-year-old woman who was admitted with a left-sided breast lump in the inner upper quadrant that was initially diagnosed as a myoepithelioma with potentially malignant disorder. At 12 months after resection, she complained about a newly developed solid mass in the subareolar region of the ipsilateral breast that was diagnosed as an invasive ductal carcinoma. In addition, 16 months after the initial admission, a re-growing remnant lesion recurred in the inner upper quadrant and was ultimately diagnosed as a myoepithelial carcinoma. Lymph node metastasis of the myoepithelial carcinoma was also observed in her left axillary region 11 months after local recurrence. A cytogenetic analysis showed recurring specific chromosomal alterations both in the locally recurrent and in the lymph-node metastatic lesion: 48, XX, t(5;18)(q13;q23),del(6)(q?),+14. + mar1. To our knowledge, this is the first published report of clonal chromosomal rearrangements in myoepithelial carcinoma of the breast that presented as metachronic double cancer with invasive ductal carcinoma in the ipsilateral breast.

DOI： 10.1016/j.cancergen.2016.10.005

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記述言語：英語   出版者・発行元：(一社)日本小児血液・がん学会  

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

A giant-cell tumor of the bone (GCTB) is a benign but locally aggressive bone tumor. Recently, the receptor activator of nuclear factor κB (RANK) ligand inhibitor, denosumab, has demonstrated activity against giant-cell tumors. The current study reports a case of a sacral GCTB with lung metastasis. A 19-year-old male patient presented with right buttock pain and right lower leg pain, and a sacral GCTB was diagnosed based on the histological analysis of a biopsy specimen. The patient was successfully treated with neoadjuvant denosumab therapy, which allowed curettage. In addition, the pulmonary nodule reduced in size following denosumab administration, and surgical resection was performed. Since the operation, the patient has been managed with the continued use of denosumab with no sign of recurrence. Microscopic findings from the surgical specimen following denosumab treatment revealed that the giant cells had disappeared and woven bone had formed. The specimen from the pulmonary nodule exhibited similar findings to the surgical specimen. It was reported that denosumab treatment was able to reduce the number of giant cells and RANK-positive stromal cells, and cause the formation of new bone in the primary lesion. The present study reports the first case to demonstrate the efficiency of denosumab in treating pulmonary metastasis of GCTB.

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

The purpose of this study is to investigate the utility of the apparent diffusion coefficient (ADC) in differentiating benign and malignant uterine smooth muscle tumors classified by signal intensity (SI) on T2-weighted imaging (T2WI) and diffusion-weighted imaging (DWI), and to determine the correlation between ADC and tumor cell density. This retrospective study reviewed 168 lesions in 134 cases with pathologically confirmed uterine smooth muscle tumors, including 6 leiomyosarcomas and 3 smooth muscle tumors of uncertain malignant potential, and preoperative magnetic resonance imaging examinations performed between October 2009 and November 2012. T2WI and DWI were also performed for each subject. Tumors were then classified according to SI on T2WI and DWI relative to myometrial SI. The correlation between ADC and tumor cell density was also determined. In Group 1 (high on both T2WI/DWI), mean ADC was significantly lower for leiomyosarcoma (0.91 × 10(-3) mm(2)/s) than for leiomyoma (1.30 × 10(-3) mm(2)/s; p < 0.05) and mean cell density significantly higher for leiomyosarcoma (42.9%) than for leiomyoma (22.4%; p < 0.05). A strong negative correlation was seen between ADC and tumor cell density in Group 1 (Spearman, R = -0.72; p < 0.05). ADC may help to differentiate benign from malignant uterine smooth muscle tumors, particularly tumors with high SI on T2WI and DWI.

DOI： 10.1007/s00261-014-0324-5

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記述言語：日本語   出版者・発行元：(NPO)日本肺癌学会  

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Soft tissue tumors arising in deep veins of the extremities are uncommon, although a few cases of synovial sarcoma or leiomyosarcoma arising in the femoral vein have been documented. However, to the best of our knowledge, an extraskeletal myxoid chondrosarcoma (EMC) arising in the femoral vein has not been reported in the English literature. We report a case of EMC arising in the femoral vein of a 70-year-old man who presented with right leg edema and was diagnosed with a deep venous thrombosis (DVT) by computed tomography (CT). Magnetic resonance imaging (MRI) revealed a mass in the right proximal thigh that was diagnosed as myxomatous sarcoma by aspiration cytology, and anticoagulant therapy was initiated. The mass was surgically resected en bloc, including the femoral vein and surrounding soft tissue, and the femoral artery was preserved. The femoral vein was not reconstructed. The histologic diagnosis was an extraskeletal myxoid chondrosarcoma. The patient received postoperative local radiation treatment, with a total dose of 60 Gy, and is currently doing well with no evidence of local recurrence or metastasis at 8 months after surgery. In summary, this case report shows that EMC can arise in the femoral vein, and that reconstruction of the femoral vein is not always necessary during surgery for soft tissue tumors.

DOI： 10.1007/s00256-014-1897-3

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1212/WNL.0000000000000714

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Diagnosing adipocytic tumors can be challenging because it is often difficult to morphologically distinguish between benign, intermediate and malignant adipocytic tumors, and other sarcomas that are histologically similar. Recently, a number of tumor-specific chromosome translocations and associated fusion genes have been identified in adipocytic tumors and atypical lipomatous tumors/well-differentiated liposarcomas (ALT/WDL), which have a supernumerary ring and/or giant chromosome marker with amplified sequences of the MDM2 and CDK4 genes. The purpose of this study was to investigate whether quantitative real-time polymerase chain reaction (PCR) could be used to amplify MDM2 and CDK4 from total RNA samples obtained from core-needle biopsy sections for the diagnosis of ALT/WDL. METHODS: A series of lipoma (n = 124) and ALT/WDL (n = 44) cases were analyzed for cytogenetic analysis and lipoma fusion genes, as well as for MDM2 and CDK4 expression by real-time PCR. Moreover, the expression of MDM2 and CDK4 in whole tissue sections was compared with that in core-needle biopsy sections of the same tumor in order to determine whether real-time PCR could be used to distinguish ALT/WDL from lipoma at the preoperative stage. RESULTS: In whole tissue sections, the medians for MDM2 and CDK4 expression in ALT/WDL were higher than those in the lipomas (P < 0.05). Moreover, karyotype subdivisions with rings and/or giant chromosomes had higher MDM2 and CDK4 expression levels compared to karyotypes with 12q13-15 rearrangements, other abnormal karyotypes, and normal karyotypes (P < 0.05). On the other hand, MDM2 and CDK4 expression levels in core-needle biopsy sections were similar to those in whole-tissue sections (MDM2: P = 0.6, CDK4: P = 0.8, Wilcoxon signed-rank test). CONCLUSION: Quantitative real-time PCR of total RNA can be used to evaluate the MDM2 and CDK4 expression levels in core-needle biopsies and may be useful for distinguishing ALT/WDL from adipocytic tumors. Thus, total RNA from core-needle biopsy sections may have potential as a routine diagnostic tool for other tumors where gene overexpression is a feature of the tumor.

DOI： 10.1186/1471-2407-14-468

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: The features of gastric submucosal cancer revealed by magnifying endoscopy have not been reported. Aim of our study was to investigate whether magnifying endoscopy could contribute to the diagnosis of submucosal invasion. PATIENTS AND METHODS: In this prospective, cross-sectional study, 197 lesions of gastric differentiated adenocarcinoma, diagnosed as mucosal cancer by conventional endoscopy, were observed by magnifying endoscopy with narrow-band imaging, paying attention to the presence of a blurry mucosal pattern and an irregular mesh pattern. After endoscopic submucosal dissection, all lesions were examined histologically and the areas of two features were estimated. RESULTS: Among the lesions examined, 177 were diagnosed histologically as mucosal cancer and 20 as submucosal cancer. Multivariate logistic regression analysis confirmed that a blurry mucosal pattern (odds ratio 12.15, 95% confidence interval 3.45-42.76, p=0.000) and an irregular mesh pattern (22.55, 4.22-120.45, p=0.000) were independent predictors of submucosal invasion. CONCLUSIONS: Narrow band imaging magnifying endoscopic features are useful for predicting submucosal invasion in gastric cancer.

DOI： 10.1016/j.dld.2013.09.003

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Secondary rhabdomyosarcoma (RMS) after treatment of osteosarcoma (OS) is rare. Reported here is the case of a metachronous RMS in the nasal cavity, developing 12 years after successful treatment of non-metastatic OS. The patient was diagnosed as having OS of the femur at 2 years of age. Chemotherapy for OS included doxorubicin (cumulative dose, 488 mg/m(2) ). No radiotherapy was given. There was no family history suggestive of cancer predisposition syndrome. At 14 years of age, alveolar RMS was diagnosed on histopathology. PAX3-FKHR fusion transcripts were detected on reverse transcription-polymerase chain reaction. Germline TP53 mutation was not seen on standard DNA sequencing. The occurrence of secondary sarcomas, in the Children's Cancer Survivor study conducted in North America, has been associated with high cumulative doses of anthracyclines, which may also have played a role in the development of RMS in the present case. In the future, novel molecular technologies might uncover genetic cancer predisposition in patients with metachronous cancers.

DOI： 10.1111/ped.12070

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Primary central nervous system lymphoma (PCNSL) expressing T-cell markers is rare, among which nasal-type extranodal NK/T-cell lymphoma is an extremely rare subtype associated with Epstein-Barr virus (EBV) infection. Here we report the clinicopathologic features of a case of EBV-associated PCNSL showing a cytotoxic T-cell phenotype. The patient, a 73-year-old woman, presented with rapidly progressive mental deterioration. Brain MRI revealed multiple lesions with swelling in the bilateral cerebral hemispheres, which were hypointense on T1-weighted images, hyperintense on T2-weighted and fluid-attenuated inversion recovery images, and slightly hyperintense on diffusion-weighted images. Biopsy specimens from the temporal region showed many medium-sized anaplastic lymphocytic cells with perivascular and angio-invasive patterns in the cortex. Immunohistochemically, the cells were positive for CD3, CD8, T-cell-restricted intracellular antigen-1 (TIA-1), granzyme B and perforin, but negative for CD56 and CD20. In situ hybridization revealed EBV-encoded RNAs in the tumor cell nuclei. A rearrangement study showed T-cell receptor γ-chain gene rearrangement with a clonal appearance. The patient died 6 months after surgery, and a general autopsy revealed no lymphoma cells outside the brain. These cellular profiles are inconsistent with those of extranodal NK/T-cell lymphoma, and have not been previously described. This case appears to represent an unusual CNS manifestation of EBV-associated T-cell lymphoma.

DOI： 10.1111/neup.12005

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記述言語：日本語   掲載種別：研究論文（学術雑誌）  

A 23-year-old woman presented with a history of repeated abdominal pain and vomiting since the 12th gestational week. Radiological studies revealed dilatation of the entire small intestine without mechanical obstruction, and dysmotility of the ileocecal region was suspected. Her symptoms continued after delivery, despite conservative treatment, and ileocecal resection was performed at age 24 years. The resected specimen showed a remarkable decrease in the density of enteric ganglion cells, therefore chronic idiopathic intestinal pseudo-obstruction (CUP) was diagnosed. Subtotal colectomy was subsequently performed at 25 years of age, but her symptoms persisted. Long-term parenteral nutrition became difficult because of catheter-related complications and her nutritional status worsened. Therefore, at age 29 she underwent jejunostomy, and her symptoms improved. She is now able to eat and her nutritional status continues to improve. Autoimmune abnormality has recently been reported to be one of the causes of CUP, and some relation between pregnancy and autoimmune disease has been recognized. Our case suggests that immunological changes during pregnancy may cause CUP. © 2012 The Japanese Society of Gastroenterological Surgery.

DOI： 10.5833/jjgs.45.326

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Nuclear factor-κB essential modulator (NEMO) deficiency is a developmental and immunological disorder. The genetic and phenotypic correlation has been described. METHODS: We report a unique clinical presentation and the identification of a novel missense mutation in the NEMO gene in a 3-year-old boy with bacillus Calmette-Guerin (BCG) infection. RESULTS: The patient presented with fever, cervical lymphadenopathy, and abnormal anti-Epstein-Barr virus (EBV) antibody titers, suggestive of EBV-related diseases including chronic active EBV infection, X-linked lymphoproliferative syndrome, or nasopharyngeal carcinoma. Although the biopsy specimen from a nasopharyngeal lesion was initially diagnosed as squamous cell carcinoma, this was changed to disseminated BCG infection involving the nasopharynx, multiple systemic lymph nodes, and brain. A novel mutation (designated D311E) in the NEMO gene, located in the NEMO ubiquitin-binding (NUB) domain, was identified as the underlying cause of the immunodeficiency. Impaired immune responses which are characteristic of patients with NEMO deficiency were demonstrated. The patient underwent successful unrelated bone marrow transplantation at 4.9 years of age. CONCLUSION: This study suggests the importance of the NUB domain in host defense against mycobacteria. The unique presenting features in our patient indicate that a hypomorphic NEMO mutation can be associated with atypical pathological findings of the epithelial tissues in patients with BCG infection.

DOI： 10.1007/s10875-011-9568-9

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記述言語：日本語   掲載種別：研究論文（学術雑誌）  

A-35-year-old female visited our hospital complaining of lower abdominal pain and atypical genital bleeding. A detailed examination revealed a rectal cancer with metastasis to the right ovary. Invasions to the uterus and right ureter were also detected. Three courses of XELOX therapy were administered as neoadjuvant chemotherapy. A subsequent examination revealed a significantly smaller metastatic lesion, and we therefore performed a radical resection. The pathological examination revealed that all surgical margins were negative for malignancy. We determined that there was no residual cancer and evaluated the surgery as curability A. The patient was administered XELOX therapy as adjuvant chemotherapy a month after the operation. Three months after the operation, the patient has remained in good condition with no signs or symptoms of tumor recurrence.

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Gastric carcinoma is one of the malignancies that are most frequently associated with esophageal carcinoma. We describe herein our device for advanced esophageal cancer associated with early gastric cancer in the antrum. A 57-year-old man presenting with dysphagia and upper abdominal pain was admitted to our hospital. Preoperative examinations revealed locally advanced squamous cell carcinoma (SCC) of the middle thoracic esophagus (T3N0M0 Stage IIA) and mucosal signet-ring cell carcinoma of the gastric antrum (T1N0M0 Stage IA). Although the gastric tumor appeared to be an intramucosal carcinoma, its margin was obscure, so endoscopic en-bloc resection was considered inadequate. We chose surgical resection of the gastric tumor as well as the esophageal SCC after neoadjuvant chemotherapy with 5-fluorouracil and cisplatin for advanced esophageal cancer. Following transthoracic esophagectomy with three-field lymph node dissection, the gastric carcinoma was removed by gastric antrectomy, which preserved the right gastroepiploic vessels, and a pedunculated short gastric tube was used as the esophageal substitute. Twenty-eight months after the surgery, the patient is well with no evidence of cancer recurrence. Because it minimizes surgical stress and organ sacrifice, gastric tube interposition is a potentially useful technique for esophageal cancer associated with localized early gastric cancer.

DOI： 10.4251/wjgo.v3.i5.75

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記述言語：英語  

A 75-year-old female, who had an abnormal stomach x-ray finding, was admitted to the hospital for further examination and therapy. Upper GI endoscopy showed reddish and swollen folds on the greater curvature of the gastric body and a biopsy was of this lesion revealed malignant lymphoma (small cell type or mucosa-associated lymphoid tissue (MALT) lymphoma suspected). The patient was infected with Helicobacter pylori (H. pylori), however, in response to the patient's wishes, a total gastrectomy, omentectomy and splenectomy were performed and the histological diagnosis was gastric MALT lymphoma. Two courses of CHOP therapy (cyclophosphamide (CPM) 750 mg/m(2)/day, day 1, adriamycin (ADM) 50 mg/m(2)/day, day 1, vincristine sulfate (VCR) 1.4 mg/m(2)/day, day 1, prednisolone 100 mg/body, day 1-5) were administered as adjuvant chemotherapy. A colonoscopic examination performed about 4.5 yr after the operation revealed rectal submucosal tumors and the biopsied specimens were diagnosed as malignant lymphoma. A transanal focal resection was performed and the histological diagnosis was metachronous and ectopic development of MALT lymphoma. The histological finding was similar to the gastric lesion. About 4 and 7 yr after the first development of rectal MALT lymphoma, MALT lymphomas developed repeatedly in the rectal lesion, however, these were resected repeatedly and no developmenthas occurred during the past two years. This report presents a very rare case of metachronous and ectopic MALT lymphoma development in the gastric and rectal lesions.

DOI： 10.4081/rt.2011.e24

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Podoplanin is a 38 kDa mucin-type transmembrane glycoprotein that was first identified in rat glomerular epithelial cells (podocytes). It is expressed in normal lymphatic endothelium, but is absent from vascular endothelial cells. D2-40 is a commercially available mouse monoclonal antibody which binds to an epitope on human podoplanin. D2-40 immunoreactivity is therefore highly sensitive and specific for lymphatic endothelium. Recent investigations have shown widespread applications of immunohistochemical staining with D2-40 in evaluating podoplanin expression as an immunohistochemical marker for diagnosis and prognosis in various tumors. To determine whether the podoplanin (D2-40) antibody may be useful for the diagnosis of soft tissue tumors, 125 cases, including 4 kinds of benign tumors, 15 kinds of malignant tumors and 3 kinds of tumor-like lesions were immunostained using the D2-40 antibody. Total RNA was extracted from frozen tumor tissue obtained from 41 corresponding soft tissue tumor patients and 12 kinds of soft tissue tumor cell lines. Quantitative real-time PCR reactions were performed. Immunohistochemical and quantitative real-time RT-PCR analyses demonstrated the expression of the podoplanin protein and mRNA in the majority of benign and malignant soft tissue tumors and tumor-like lesions examined, with the exception of alveolar soft part sarcoma, embryonal and alveolar rhabdomyosarcoma, extraskeletal Ewing's sarcoma/peripheral primitive neuro-ectodermal tumor and lipoma, which were completely negative for podoplanin. Since it is widely and highly expressed in nearly all kinds of soft tissue tumors, especially in spindle cell sarcoma, myxoid type soft tissue tumors and soft tissue tumors of the nervous system, podoplanin is considered to have little value in the differential diagnosis of soft tissue tumors.

DOI： 10.3892/or.2011.1141

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

AIMS: To investigate TTF-1-negative pulmonary adenocarcinoma, focusing upon mucin production and the expression of hepatocyte nuclear factor-4α (HNF4α). MATERIALS AND METHODS: Two hundred and sixty-two cases of pulmonary adenocarcinoma were examined histologically and immunohistochemically; TTF-1 was expressed in 222 cases (84.7%), and 40 cases (15.3%) were negative. Among TTF-1-negative cases there were 31 mucinous-type tumours, and HNF4α, MUC5AC and MUC2 were expressed in 34 cases (85%), 29 cases (72.5%) and four cases (10%), respectively. In contrast, their expression was rare in TTF-1-positive tumours. A statistically inverse correlation was confirmed between the expression of TTF-1 and that of HNF4α and MUC5AC. CONCLUSION: Most TTF-1-negative pulmonary adenocarcinomas are mucinous lesions with the predominant expression of HNF4α and MUC5AC.

DOI： 10.1111/j.1365-2559.2011.03764.x

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1007/s00776-010-0001-7

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: FUS1 is a tumor suppressor gene located on human chromosome 3p21.3. Frequent loss of FUS1 protein expression is associated with lung cancer development. This study examined FUS1 expression and its possible tumor-suppressive role in bone and soft tissue sarcomas. MATERIALS AND METHODS: The expressions of FUS1 mRNA and FUS1 protein were assessed in sarcoma cell lines, sarcoma tissues, benign bone and soft-tissue tumor (BST) tissues, and healthy tissues. Exogenous FUS1 gene transfection was performed on sarcoma cell lines. RESULTS: FUS1 mRNA expression was detected in all sarcoma cell lines, all benign BSTs and healthy tissues, and almost all sarcoma tissues. In contrast, FUS1 protein expression was frequently lost in sarcoma cells and sarcoma tissues. The exogenous FUS1 gene delivery induced strong FUS1 protein expression, inhibition of cell viability and apoptosis in sarcoma cells. CONCLUSION: FUS1 may act as a tumor suppressor in bone and soft-tissue sarcomas.

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記述言語：日本語   出版者・発行元：(公社)日本整形外科学会  

表在性の軟部腫瘍や骨外性病変が触知できる骨腫瘍では触診しながら腫瘍または骨外病変を直接穿刺し、通常の骨腫瘍はX線透視下に穿刺を行った。深部の骨・軟部腫瘍に対してはCTガイド下に穿刺を行った。骨腫瘍および軟部腫瘍に対して行われた針生検983例のうち、最終的に切除材料または開放生検で確認ができた穿刺吸引細胞診(FNAC)359例(骨腫瘍90例、軟部腫瘍269例)、針組織生検(CNB)127例(骨腫瘍40例、軟部腫瘍87例)を対象とした。FNACでは、誤診はすべて偽陰性で3例に認めた。感度、特異度、偽陽性率、偽陰性率はそれぞれび94.3%、100%、0%、5%で、正診率は96.7%であった。CNBでは、臨床所見より判定が正しいものと判断された。誤診例はなかった。感度、特異度、偽陽性率、偽陰性率はそれぞれ100%、100%、0%、0%であり、正診率は100%であった。

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Thymic carcinoma is a rare tumor. The most common histological subtype is squamous cell carcinoma, and only a few cases of thymic adenocarcinoma have been reported. A case of papillary adenocarcinoma of thymic origin that coexisted with type AB thymoma as a separate nodule is presented herein. The patient was found to have an abnormal mediastinal shadow on chest X-ray. A computed tomography scan revealed a round, 6.5-cm-diameter mass in the right anterior mediastinum. The preoperative diagnosis was thymoma, and thymothymectomy was performed. On pathological examination, two tumors, which were diagnosed as papillary adenocarcinoma and type AB thymoma, respectively, were present in the thymus without any connection with each other. The patient has been alive without any signs of recurrence for 11 years after surgery. We diagnosed the adenocarcinoma in this case was a primary thymic carcinoma.

DOI： 10.1007/s11748-009-0580-4

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Podoplanin is known as a lymphatic marker because its expression is detected in lymphatic but not vascular endothelium. Podoplanin is also expressed in several normal tissues including osteocytes or osteoblasts. A systematic examination of the podoplanin expression was conducted in normal skeletal tissues and some bone tumor cell lines, and the diagnostic value determined in primary bone tumors. Podoplanin mRNA was expressed at a high level in bone marrow tissue and cartilage, and was upregulated with differentiation to osteoblasts in bone marrow cells. Strong podoplanin expression was seen in osteocytes, chondrocytes, and osteoblasts on immunohistochemistry. Podoplanin mRNA was expressed at a high level in several osteosarcoma and chondrosarcoma cell lines, whereas podoplanin was expressed at a low level in a Ewing's/primitive neuroectodermal tumor cell line. In the clinical samples, osteosarcomas (22/26) expressed podoplanin at various levels. In small cell osteosarcomas (2/2), podoplanin was expressed strongly, although the tissue samples included few diagnostic osteoids. Chondrosarcomas (10/10) expressed podoplanin strongly, and chondroblastomas (5/5) expressed podoplanin moderately, while podoplanin was absent or expressed at low levels in Ewing's sarcomas (0/5), chordomas (0/6) and giant cell tumors of bone (1/7). Therefore, podoplanin may be a sensitive immunohistochemical marker of osteogenic and chondrogenic bone tumors.

DOI： 10.1111/j.1440-1827.2009.02510.x

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

PURPOSE: We report here, our experience of seven patients with epithelioid sarcomas and their serum CA 125 levels, as well as the results of an in vitro and in vivo study of CA 125 expression in epithelioid sarcoma cells and xenografts using three epithelioid sarcoma cell lines. METHODS: In the clinical study, the serum CA 125 levels of seven epithelioid sarcoma patients were examined at multiple time points. Expression of the MUC16 gene that encodes the CA 125 sequence was examined using RT-PCR methods in three epithelioid sarcoma cell lines, FU-EPS-1, SFT-8606 and NEPS, and the CA 125 protein in each cell lysate was examined by Western blot using anti-CA 125 clone OC125 antibody. The concentration of CA 125 in the conditioned medium of each cell line was also measured. RESULTS: In five of the seven epithelioid sarcoma patients, CA 125 levels reflected regression and progression of their disease. The CA 125 concentrations in the conditioned medium of FU-EPS-1, SFT-8606 and NEPS cells were 259, 252, and 6 U/ml, respectively. Strong expression of MUC16 mRNA was shown in FU-EPS-1 and SFT-8606 cells: correspondingly, a thick band was observed by Western blot analysis in only FU-EPS-1 and SFT-8606 cells. CONCLUSION: We concluded that epithelioid sarcoma cells produce and secrete CA 125 into the blood serum and that the elevation of serum CA 125 correlates with disease progression. Therefore, measuring the serum CA 125 level should provide an useful index for diagnosing and monitoring the course of epithelioid sarcoma.

DOI： 10.1007/s00432-009-0678-1

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Thymic carcinoma is a rare tumor. Limited data are available regarding the effectiveness of treatment and the prognosis of thymic carcinoma. The present study aimed to clarify the prognostic factors in patients who underwent resection. METHODS: The study retrospectively reviewed 21 patients (15 men, 6 women) with thymic carcinoma who had undergone resection at Niigata University Hospital. RESULTS: Masaoka stage was II in 4 patients, III in 9, IVa in 2, and IVb in 6. Histologic subtypes were squamous cell carcinoma in 14 patients, adenocarcinoma in 2, atypical carcinoid in 3, and undifferentiated carcinoma in 2. Histologic grade by degree of differentiation was low in 4 tumors, intermediate in 12, and high in 5. Treatment comprised resection alone in 6 patients and resection along with multimodal therapies in 15. Complete resection was achieved in 14 (67%). Eight patients died of tumor. Recurrence was documented in 7 of 14 patients with complete resection, and 5 received additional treatment. The overall 5-year survival rate was 61.1%, and the disease-free 5-year survival rate was 66.8% for the 14 with complete resection. By multivariate analysis, Masaoka stage and histologic grade were significant independent prognostic factors for overall survival. CONCLUSIONS: The surgical outcome of patients with thymic carcinoma depends on the Masaoka stage and histologic grade. Patients with early Masaoka stage and low or intermediate histologic grade had favorable prognoses.

DOI： 10.1016/j.athoracsur.2009.11.057

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

As a result of having undergone computed tomography (CT), a 75-year-old woman with type-C liver cirrhosiswas shown to have two tumors on the ventral and dorsal sides of subsegment 3 (S3). The tumor on the ventral side was diagnosed as a classic hepatocellular carcinoma (HCC), while that on the dorsal side was considered atypical for a HCC. Although the indocyanine green (ICG) findings indicated poor hepatic reserve, the prothrombin time (PT) was relatively good. An operation was performed in February 2007; however, this resulted in exploratory laparotomy. Dynamic CT performed 12 mo after the operation revealed that the tumor on the dorsal side of S3 had apparently increased. The marginal portion of the tumor was shown to be in the early and parenchymal phases, while the internal portion was found to have grown only slightly in the delayed phase. We diagnosed this tumor as a cholangiocellular carcinoma (CCC). S3 subsegmentectomy was performed in April 2008. The tumor on the ventral side was pathologically diagnosed as a moderately differentiated HCC, and that on the dorsal side was diagnosed as a CCC. We can therefore report a rare case of synchronous development of HCC and CCC in the same subsegment of the liver in a patient with type-C liver cirrhosis. We also add a literature review for all the reported cases published in Japan and around the world, and summarize the features of double cancer exhibiting both HCC and CCC.

DOI： 10.4254/wjh.v1.i1.103

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Rosai-Dorfman disease (RDD) is a rare condition of unknown etiology, and female genital tract involvement in RDD is uncommon. We describe the first case of RDD with bilateral ovarian involvement in a patient implanted with a ventriculoperitoneal (VP) shunt. The patient was a 17-year-old Japanese woman who had undergone radiotherapy, surgery for extranodal RDD involving the brain, and VP shunt insertion at age 12. Bilateral pelvic masses were incidentally detected on a computed tomography scan. She underwent laparotomy for lesion extirpation. On abdominal washing cytology, histiocytes showing emperipolesis were identified. Bilateral salpingo-oophorectomy was performed instead of extirpation, as it was difficult to identify the lesion margins. At 24 months after surgery, the patient is well and has not developed local recurrence. Thus, RDD can recur because of implantation of lesion cells into the abdominal cavity through a VP shunt, as is observed in the case of cerebral neoplasms.

DOI： 10.1111/j.1447-0756.2009.01054.x

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Pleomorphic rhabdomyosarcoma (PRMS) is a rare variant of rhabdomyosarcoma that occurs mostly in adults. A few cytogenetic studies of PRMS have been reported, but no consistent specific chromosome aberrations were detected. We herein report a cytogenetic study of three cases of pleomorphic rhabdomyosarcoma using a conventional G-banded karyotyping analysis. The three cases appeared to exhibit an extremely complex karyotype with numeric and structural rearrangements. Although the three cases displayed several common aberrations, including -2, -4, -9, -13, -14, -15, -19, -21, add(X)(p11), add(1)(q11), add(7)(p11), and add(13)(p11), no recurrent characteristic chromosomal aberrations could be detected. In addition, among these cases and seven other cases of previously reported PRMS, the most frequent chromosomal alterations were -2, -13, -14, -15, -16, and -19. No obviously consistent structural alterations can be found in these 10 PRMS cases, however, thereby suggesting that it is difficult to confirm whether these complex karyotypes correlated with the diagnosis or clinical outcome in PRMS. In this study, we detected MyoD1 and myogenin gene transcripts at the mRNA level in four cases of PRMS together with other soft-tissue sarcomas, including seven cases of malignant fibrous hitiocytoma, five cases of liposacroma, and three cases of leiomyosacroma using a real-time quantitative reverse-transcriptase polymerase chain reaction (RT-PCR) analysis. High-level expressions of MyoD1 and myogenin gene transcripts were determined in all cases of PRMS. In contrast, the other non-PRMS sarcomas showed either no expression or extremely weak expressions for both genes. Our findings suggest that the detections of MyoD1 and myogenin transcripts using real-time quantitative RT-PCR, combined with immunohistochemical stains, are extremely sensitive and useful for the diagnosis of PRMS.

DOI： 10.1016/j.cancergencyto.2009.02.011

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Alveolar soft part sarcoma (ASPS) is a distinct, rare soft tissue tumor with an unknown histogenesis and a tendency for late widespread metastases to lung, bone, and brain. It is now clear that they are caused by a specific unbalanced translocation, der(17)t(X;17)(p11;q25), which results in the formation of an ASPSCR1-TFE3 (alias ASPL-TFE3) fusion gene. The rearrangement results in the expression of chimeric transcripts, which can be identified by means of reverse transcriptase-polymerase chain reaction (RT-PCR). We investigated the histogenesis of ASPS and attempted to detect circulating ASPS tumor cells in peripheral blood. The immunohistochemical and genetic details of four cases and one cell line of ASPS were examined. An immunohistochemical analysis and RT-PCR did not detect myogenic differentiation gene MYOD1. The sensitivity of nested RT-PCR for detection of circulating ASPS cells was assessed by demonstrating that the tumor cell-associated gene translocation could be detected in 50 tumor cells/2 mL of blood. Clinically, it was detectable in a peripheral blood sample (2 mL) of ASPS patient with distant metastases. The findings suggest that ASPS is not of skeletal muscle origin. ASPS tumor cells in the peripheral blood could be monitored by RT-PCR.

DOI： 10.1016/j.cancergencyto.2008.11.014

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

The enzymes thymidylate synthase (TS), dihydropyrimidine dehydrogenase (DPD) and orotate phosphoribosyl transferase (OPRT) are involved in the metabolism of the anticancer drug 5-fluorouracil (FU). Expression of TS, DPD and OPRT in cancer tissue has been reported to be associated with sensitivity and/or resistance to 5-FU therapy. However, the role of TS, DPD and OPRT expression in lung cancer has not been fully established. Furthermore, among several measuring methods, it is not clear which method effectively predicts the response to 5-FU therapy. The aim of this study was to analyze the expression of 5-FU-related enzymes using enzyme-linked immunosorbent assay (ELISA) and to examine the correlation of ELISA and the results obtained using different measuring methods such as reverse transcript polymerase chain reaction (RT-PCR), immunohistochemistry, and enzymatic activity. Lung cancer specimens were obtained from 134 patients who underwent curative resection for lung cancer. As a pilot study, enzyme expression of 11 samples was measured using 4 different methods for DPD: RT-PCR, immunohistochemistry, enzymatic activity and ELISA. The relationships between pairs of results were compared, and then enzyme protein expression was measured using ELISA in 119 patients with adenocarcinoma. Of the 4 independent methods, the highest correlation was observed between protein expression measured by ELISA and enzyme activity. The correlation of gene expression and ELISA was also significant. The protein level in stage I adenocarcinoma measured using ELISA was 13.0+/-24.8 ng/mg protein for TS, 362.2+/-264.3 ng/mg protein for DPD and 4.5+/-2.0 ng/mg protein for OPRT. The predictive value of the enzymes for prognosis and the effectiveness of 5-FU was not determined as few recurrences were observed during the short follow-up period. In conclusion, ELISA is a simple and reliable method to measure key enzymes related to 5-FU therapy.

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Multinucleated cells are frequently seen in association with a malignant neoplasm. Some of these multinucleated cells are considered to be neoplastic. The mechanism of neoplastic multinucleation remains unknown, but is considered to be induced by either cell-cell fusion or acytokinetic cell division. Myxofibrosarcoma consists of spindled and pleomorphic tumor cells and bizarre multinucleated giant cells. Some of these multinucleated cells are considered to be neoplastic. METHODS: We studied the mitotic activity of the multinucleated cells by Ki-67 immunohistochemistry, and the dynamics and differentiation by live-cell video microscopy in the two myxofibrosarcoma cell lines to determine whether the mechanism of multinucleation is cell-cell fusion or acytokinetic cell division RESULTS: A Ki-67 immunohistochemical analysis revealed a high positive rate of multinucleated cells, as well as mononuclear cells, and mitotic ability was shown in the multinucleated cells. In live-cell video microscopy, most of the multinucleated cells were induced via the process of acytokinetic cell division. CONCLUSION: The current study indicates that a vulnerability of the cytoskeleton components, such as the contractile ring, causes multinucleation to occur from the telophase to the cytokinesis of the cell cycle.

DOI： 10.1186/1756-9966-28-44

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

OBJECTIVE: To assess the relationship between in vitro chemosensitivity evaluated by the histoculture drug response assay (HDRA) and the expression of beta-tubulin isotypes in tumors of patients with completely resected NSCLC in order to determine the predictive value of beta-tubulin in chemotherapy for NSCLC. METHODS: Expression of beta-tubulin isotypes was immunohistochemically analyzed in a series of 58 tumor samples from patients with completely resected NSCLC. The sensitivity of individual tumors to anticancer agents was evaluated by HDRA. RESULTS: Class III beta-tubulin expression by tumor cells was significantly correlated with resistance to docetaxel (p=0.0250), but not related with resistance to gemcitabine. Patient characteristics (age, gender, histology, and stage) were not associated with class III beta-tubulin expression. CONCLUSION: An abundance of class III beta-tubulin in tumor cells could be a biomarker for resistance to docetaxel in patients with completely resected NSCLC.

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Esophageal schwannoma is rare and it is difficult preoperatively to confirm a definitive diagnosis, even using current imaging techniques. We present a case of a benign esophageal schwannoma that was surgically excised and confirmed by immunohistochemical staining. Conventional radiological studies, including barium meal, computed tomography and endoscopic examination had shown a solid submucosal tumor of the upper thoracic esophagus but had been unable to confirm the diagnosis. Positron emission tomography was carried out to evaluate the malignant potential and showed a high uptake of 18F-fluorodeoxyglucose (FDG) into the tumor in both the early and delayed phase, suggesting that the tumor was a potentially malignant tumor such as a gastrointestinal stromal tumor. This is the first reported case of esophageal schwannoma that indicated a high FDG uptake. Although consensus has not been reached regarding the precise mechanism of FDG accumulation in schwannomas, we discuss our clinicopathological findings and review other studies of the subject.

DOI： 10.1111/j.1442-2050.2007.00712.x

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Overexpression of KIT, a tyrosine kinase receptor protein encoded by the proto-oncogene c-kit, is observed in human neoplasms such as gastrointestinal stromal tumors (GISTs), myeloproliferative disorders, melanoma and seminoma. In patients with GIST, overexpression of mutated KIT within the tumor is predictive of response to molecular targeted therapy using imatinib. However, the role of KIT expression in thymic carcinoma is not fully understood. METHODS: Thymic epithelial tumors from 37 patients (17 thymic carcinomas and 20 thymomas) were examined. Immunohistochemical staining with anti-KIT polyclonal antibody and anti-CD5 was performed. Mutation analyses in the juxtamembrane domains, exons 9 and 11, and in the tyrosine kinase domains, exons 13 and 17, were undertaken using polymerase chain reaction (PCR) and direct DNA sequencing in KIT-positive samples. RESULTS: KIT- and CD5-positive staining was observed only in thymic carcinoma. Percentage of positive staining was 100% in squamous cell carcinoma, with no positive staining in other histologies, including atypical carcinoid. Mutation analysis of the KIT gene was performed in 11 squamous cell carcinomas, 1 adenocarcinoma and 1 adenosquamous cell carcinoma. None of the tested samples showed mutations in any of the four exons. CONCLUSIONS: Squamous cell carcinoma of the thymus frequently expressed KIT and CD5 proteins, whereas other tumors did not. Unlike GIST, overexpression of KIT does not necessarily indicate gene mutation in thymic carcinoma. KIT and CD5 appear useful for evaluating and subtyping thymic epithelial tumors.

DOI： 10.1016/j.lungcan.2008.03.035

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Hepatocyte nuclear factor 4 alpha (HNF4 alpha) is a member of the nuclear receptor superfamily and is expressed in several endodermal tissues. The aim of the present study was to examine the expression of HNF4 alpha on ovarian epithelial tumors with immunocytochemistry and immunohistochemistry using mAbs recognizing P1 and P2 promoter-driven HNF4 alpha. Ovarian mucinous adenoma, mucinous tumors of borderline malignancy, and mucinous adenocarcinoma had positive nuclear staining for HNF4 alpha (41/45, 91%). One-third (34%) of mucinous tumors had P1-positive staining and most had P1/P2-positive staining (93%). MUC2- and MUC5AC-positive staining was observed in 34% and 95% of mucinous tumors, respectively. The histological subtype of these mucinous tumors was not correlated with HNF4 alpha expression. On cytology it was found that cancer cells in the ascites from ovarian mucinous adenocarcinomas were HNF4 alpha positive, but tumor cells in ascites from other types of ovarian carcinomas were negative for HNF4 alpha. Thus, HNF4 alpha is demonstrated to be a useful marker for histological and cytological diagnosis of ovarian mucinous tumors.

DOI： 10.1111/j.1440-1827.2008.02293.x

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

We previously reported that macrophage colony-stimulating factor (M-CSF, CSF-1) played important roles in the process of the repopulation of Kupffer cells after their elimination by administration of liposome-entrapped dichloromethylene diphosphonate (lipo-MDP). In this study, we examined the repopulation of Kupffer cells and splenic red pulp macrophages in osteopetrotic (op/op) mice defective in the production of functional M-CSF and their littermate mice by using the lipo-MDP model. In untreated op/op mice, numbers of F4/80-positive Kupffer cells in the liver and F4/80-positive splenic red pulp macrophages were reduced. Repopulation of Kupffer cells and splenic macrophages was observed in littermate (op/+) mice liver by 14 days after depletion. However, in op/op mice, repopulation of Kupffer cells was not observed in Kupffer-cell-depleted op/op mice until 56 days after depletion, whereas splenic red pulp macrophages repopulated and recovered to the level of control op/op mice by 10 days after depletion. Single injection of M-CSF was effective for the induction of the repopulation of Kupffer cells, and daily administration of M-CSF induced remarkable repopulation and maturation of Kupffer cells and proliferation of macrophage precursor cells in the liver of Kupffer-cell-depleted op/op mice. These results suggest that Kupffer cells are completely M-CSF-dependent tissue macrophages, whereas splenic red pulp macrophages are composed of M-CSF-dependent macrophages and M-CSF-independent macrophages. This mouse model provides a useful tool for the study of effects of growth factor on Kupffer cell differentiation in vivo.

DOI： 10.1007/s00441-008-0586-8

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

A 65-year-old woman with liver injury was referred to our hospital in 1992. She was diagnosed with primary biliary cirrhosis (PBC) of Scheuer's histological classification stage IV. She was treated with 600 mg/day of ursodeoxycholic acid. A 1-cm mass in S7 was detected in August 1995. The serum alpha-fetoprotein (AFP) level increased to 1288 ng/mL in January 1996. Angiography showed a cotton wool-like appearance in the delayed phase. Because the size of the tumor appeared to be increasing and the serum AFP levels increased with high levels of L3 fraction, a pelioid-type hepatocellular carcinoma (HCC) was strongly suspected. Hepatic artery infusion with SMANCS and partial resection of S7 and S8 of the liver were performed in March 1996. The pathological diagnosis for theresected liver tumor was pelioid-type HCC. The serum AFP level decreased to 50 ng/mL after the operation, but relapsed HCC was detected in S6 and S7. Angiography in September 1996 revealed multiple hypervascular lesions, and hepatic artery infusion with SMANCS was again performed; however, we were unable to suppress the progression of the relapsed HCC. The patient died due to an intra-abdominal rupture of relapsed HCC and subsequent liver failure in December 1996. We report a rare case of pelioid-type HCC with numerous eosinophilic infiltrations arising from PBC.

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Dedifferentiated chondrosarcoma is a rare, highly malignant variant of chondrosarcoma in which a high-grade sarcoma coexists with a low-grade chondroid tumor. We herein review a case of dedifferentiated chondrosarcoma with an osteosarcoma omit component that occurred in the distal femur of a 38-year-old man. We established the cell line (NDCS-1) from a pleural effusion of the metastatic lung tumor. The cell line was characterized by a the G-banded karyotype, polymerase chain reaction (PCR) single-strand conformation polymorphism analysis, spectral karyotyping, and reverse transcriptase PCR (RT-PCR). The tumor exhibited complex karyotypes and a high frequency of chromosomal amplication with p53 mutation. This tumor revealed an osteoblastic and chondroblastic character in vitro and in severe combined immunodeficiency mice. The expression and phosphorylation of platelet-derived growth factor receptor-beta, which seemed to play a major role in the malignant phenotype of chondrosarcoma, was confirmed by RT-PCR and Western blotting. To our knowledge, this is the first report of the establishment of a human dedifferentiated chondrosarcoma.

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

We report on a case of a solitary fibrous tumor that developed in the thigh of an 82-year-old woman. The tumor was composed of areas of high-grade sarcoma and typical solitary fibrous tumor. Its karyotype was: 70,XXX,+X[4],+1[2],add(1)(p36)[4],add(1)[2],+2[4],-3[4],+6[4],add(6)(p11)x2[4],+7[4],+9[3],-11[4],-12[4],-13[4],add(13)(p11)x2[4],-14[4],+15[4],-16[3],-17[4],-19[4],+20,[4],+21[4],+22[2],+mar1x2[4][cp4].

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Ossifying fibromyxoid tumor (OFMT) is a rare but morphologically distinctive soft-tissue tumor. The histologic origin of this tumor is not clearly known, but its various features suggest a schwannian, neuronal, or chondroid origin. We herein report a case of a typical OFMT that occurred in the shoulder of a 65-year-old man. The karyotype exhibited the following complex numeric and structural aberrations: 42 approximately 46,XY,-Y,add(1)(q42),add(6)(p21),t(10;18)(q26;q11),der(11)t(11;15)(q23;q15),add(12)(q13),ins(14;?)(q13;?),-15,+mar. Combined with several previously reported studies, these aberrations could not identify a common cytogenetic abnormality in OFMT.

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掲載種別：研究論文（学術雑誌）   出版者・発行元：Ovid Technologies (Wolters Kluwer Health)  

DOI： 10.1097/01.rct.0000243451.25986.10

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Liver metastases are the most critical prognostic factors for patients with colorectal carcinomas (CRC). It has been reported that the dysregulation of hepatocyte nuclear factor-4alpha (HNF4alpha) expression is linked to the development of CRC, gastric cancer and hepatocellular carcinoma. The purpose of the present paper was to examine the P1 and P2 promoter-driven HNF4alpha (P1 and P2) expression in surgically resected CRC. Immunohistochemically, P1, P2, MUC1 and CD10 expression were evaluated in 63 cases of primary CRC. Positive staining with P1, P2, MUC1 and CD10 antibodies were observed in 37 (59%), 63 (100%), 42 (67%) and 27 (43%) cases, respectively. Loss or decreased P1 expression was observed with respect to the depth of the tumor invasion. The frequency of P1-positive expression in Dukes' C and D tumors was significantly lower than that in Dukes' A and B tumors. There was a relationship between the loss of P1 expression and metachronous liver metastases, and the survival rate of the P1-negative patients without liver metastasis at the time of the primary CRC resection tended to be worse than that of the P1-positive patients. These findings suggest that downregulation of P1 expression is involved in tumor metastasis and a worse prognosis.

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

The aim of the study was to examine the chronological histology of osteoinduction of highly purified beta-tricalcium phosphate (beta-TCP) implanted in dog dorsal muscles. Specimens were harvested on days 14, 28, 42, 56, 112 and 168 after implantation, and were analyzed by hematoxylin and eosin (HE) staining, tartrate-resistant acid phosphatase (TRAP) staining, immunohistochemistry, in situ hybridization, and silver impregnation. After day 28, abundant TRAP- and cathepsin K-positive multinucleated cells adhered to beta-TCP, suggesting that these cells are osteoclasts that can resorb beta-TCP. On day 56, new bone was formed and alpha1 chain of type I procollagen mRNA-positive osteoblasts lined the newly formed bone. Silver impregnation showed abundant collagen fibrils within the beta-TCP micropores. These results suggest that micropores function as a storage space for extracellular matrix components, including collagen. Newly formed bone never degenerated in the late stage, suggesting that beta-TCP has good biocompatibility and this material retains the conditions appropriate for osteointegration and bioresorption. In conclusion, beta-TCP has osteoinductivity after implantation in dog dorsal muscles without use of bone marrow cells or osteoinductive cytokines. The appearance of a large number of active osteoclasts precedes new bone formation.

PubMed

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記述言語：日本語   掲載種別：研究論文（学術雑誌）  

A 63-year-old woman was admitted to our hospital with a 1-month history of visual field defect and reduced bilateral visual acuity. Neurological examination revealed enlargement of bilateral central scotoma and blurred vision, and brain MRI showed masses in the optic chiasma and right hypothalamus on Ti-weighted image with contrast enhancement. Although the patient was treated with high-doses of methylprednisolone four times, the masses spread to the right internal capsule and the vicinity of the right lateral ventricle. An open biopsy was performed and microscopic examination of the sample revealed lymphoplasmacytic lymphoma. Although primary central nervous system lymphoma (PCNSL) developing from the optic chiasma and hypothalamus is rare, PCNSL should be considered in patients with multiple lesions in the optic chiasma and hypothalamus.

PubMed

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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  

Hepatocyte nuclear factor-4 alpha (HNF4 alpha) exists in multiple isoforms that are generated by alternative promoter (PI and P2) usage and splicing. Here we establish monoclonal antibodies (mAbs) for detecting P1 and P2 promoter-driven HNF4 alpha, and evaluate their expression in normal adult human tissues and surgically resected carcinomas of different origins. Using immunohistochemical analysis, we demonstrate that, while P1 promoter-driven HNF4 alpha is expressed in hepatocytes, small intestine, colon, kidney and epididymis, P2 promoter-driven HNF4 alpha is expressed in bile duct, pancreas, stomach, small intestine, colon and epididymis. Altered expression patterns of PI and P2 promoter-driven HNF4 alpha were observed in gastric, hepatocellular and colorectal carcinomas. HNF4 alpha was expressed in lung metastases from renal cell, hepatocellular and colorectal carcinoma but was not observed in lung tumours. The P1 and P2 promoter-driven HNF4 alpha expression pattern of tumour metastases correlated with the primary site of origin. P1 promoter-driven HNF4 alpha was also found in intestinal metaplasia of the stomach. These data provide evidence for the tissue distribution of P1 and P2 promoter-driven HNF4 alpha at the protein level and suggest that HNF4 alpha may be a novel diagnostic marker for metastases of unknown primary. We propose that the dysregulation of alternative promoter usage of HNF4 alpha is associated with the pathogenesis of certain cancers. Copyright (c) 2006 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

DOI： 10.1002/path.1928

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Prominent osteoconductive activity and the biodegradable nature of commercially available beta-tricalcium phosphate (beta-TCP, OSferion) have been documented in animal experiments. We analyzed four cases of involving grafted OSferion in human bone with respect to histological features by routine hematoxylin and eosin staining, silver impregnation, immunohistochemistry and in situ hybridization. OSferion affords early bioresorption by osteoclasts, vascular invasion of macropores and osteoblastic cell attachment on the surface on the ceramic surface 14 days after grafting. Prominent bone formation and direct bone connection between preexisting bone and OSferion were evident 28 days after grafting. Nearly the entire TCP surface was covered by lamellar bone; additionally, active osteoblastic lining and attachment of the osteoclast-like giant cells were not observed 72 weeks after grafting. Silver impregnation revealed the presence of collagen fibrils within probable micropores of OSferion.

PubMed

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掲載種別：研究論文（学術雑誌）   出版者・発行元：Ovid Technologies (Wolters Kluwer Health)  

DOI： 10.1097/01.rct.0000172672.09564.8c

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

We established a novel human myxofibrosarcoma cell line NMFH-1 and analyzed it with spectral karyotyping and comparative genomic hybridization (CGH). NMFH-1 cells are composed of two different types of cells, small, spindle-shaped mononuclear cells and bizarre multinucleated giant cells, which were maintained in vitro over 200 passages. Xenografted tumor showed typical features of myxofibrosarcoma, which included bizarre multinucleated giant cells. Cytogenetic analyses revealed complex abnormalities, including a t(17;22)(q2?2;q13), which has been found in dermatofibrosarcoma protuberans. Subsequent reverse-transcription polymerase chain reaction revealed that the cell line did not have the COL1A1-PDGFB gene fusion. Significant gains of the 1q12 approximately q23 and 8q13 approximately qter regions and loss of the 9p21 approximately pter and 13q12 regions often found in MFH were observed by CGH analysis. We investigated the origin of multinucleated giant cells in xenografted tumor through DNA in situ hybridization. In this system, the human-specific Alu sequence and the mouse L1 sequence were used as specific cell markers of identity. In situ hybridization revealed neoplastic proliferation of the multinucleated giant cells of human origin.

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Subcutaneous leiomyosarcoma is a rare condition that accounts for 1% to 2% of all superficial soft tissue malignancies. Approximately 10% of cases arise in the trunk, although the extremities are the most commonly affected. CASE PRESENTATION: We report herein the case of a 31-year-old man with a subcutaneous leiomyosarcoma, measuring 124 x 105 mm, arising in the left inguinal region. A wide local excision (with a resection margin >/= 20 mm) was performed. Histological examination of the resected specimen revealed a leiomyosarcoma with high cellularity and two mitoses per 10 high-power fields. The patient remains well with no evidence of disease 5 years and 8 months after the operation. CONCLUSION: This is the first reported case of subcutaneous leiomyosarcoma arising in the inguinal region and also one of the largest tumors reported. The experience of this case and a review of the English-language literature (PubMed, National Library of Medicine, Bethesda, MD, USA) suggest that a resection margin of >/= 10 mm is recommended when excising this rare tumor.

PubMed

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記述言語：日本語   掲載種別：研究論文（学術雑誌）  

A 6-year-old boy presented with headache and vomiting. Brain and spinal MRI demonstrated a large mass in the cerebellar vermis and 4th ventricle and showed thick spinal subarachnoid dissemination. Suboccipital craniotomy was performed and the tumor was totally removed. The histological diagnosis was medulloblastoma. The patient subsequently received craniospinal irradiation, and also received systemic and intrathecal perfusion chemotherapy. Then complete remission was achieved 10 months after operation. Three years later, however, a swelling at the left mandibular angle appeared. A CT scan revealed osteosclerotic lesion. After biopsy was performed, the specimen was detected infiltration of medulloblastoma cells. Bone scintigram showed a single lesion and MRI of brain and spinal cord revealed no recurrence of the central nervous system lesions. He underwent local irradiation and systemic chemotherapy with ICE regimen. This is the rare case of extracranial metastasis following remission of medulloblastoma in childhood.

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Sclerosing epithelioid fibrosarcoma is a recently described, rare mesenchymal neoplasm. We report a case of sclerosing epithelioid fibrosarcoma that occurred in the lower leg of a 48-year-old man. The karyotype of the tumor exhibited der(1)t(1;10)(p31;p11), der(10)t(10;17)(p11;q11), and der(17) t(11;17)(?;q11). Rearrangement of 10p11 was also found in one previous reported case of this uncommon tumor.

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

OBJECTIVE: To distinguish between benign and malignant peripheral nerve sheath tumors around the pelvis. METHODS: A retrospective study of 30 patients with benign and malignant peripheral nerve sheath tumors located around the pelvis was performed. Clinical, imaging and histological features of 19 benign and 11 malignant peripheral nerve sheath tumors around the pelvis were reviewed retrospectively. RESULTS: Nearly all patients exhibited pain at presentation in cases involving both benign and malignant tumors. Although tumor size, duration of symptoms and presence of sensory disturbance possessed little value in differential diagnosis, severe motor weakness was observed exclusively in patients presenting with malignant tumors. On CT or MRI, central enhancement was apparent in 11 of the 19 benign tumors; in contrast, central enhancement was evident in one of the 11 malignant tumors. Fine needle aspiration cytology was performed in 11 tumors; correct diagnosis was achieved in four tumors. Core needle biopsy was performed in five tumors, all of which were correctly diagnosed with no neurological deficits. Immunohistochemically, all benign tumors were diffusely positive for S-100 protein, whereas malignant tumors were negative or focally positive for S-100 protein. Ki-67 index was less than 4% in all benign tumors; additionally, this index was 7-36% in malignant tumors. CONCLUSION: Central enhancement pattern on imaging studies strongly suggests a benign tumor; in contrast, severe motor weakness suggests malignant lesions. Core needle biopsy was reliable with respect to preoperative diagnosis.

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

We report a case of plexiform schwannoma located in the flexor muscles of the forearm in the absence of other signs of neurofibromatosis or schwannomatosis. Magnetic resonance examination revealed a multinodular irregular inhomogeneous mass. Some nodules displayed a peripheral, high intensity rim and a central low intensity (target sign) on T2-weighted images. Pre-operative diagnosis of the rare plexiform schwannoma may be possible with careful imaging examination for the target sign.

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Liposclerosing myxofibrous tumor (LSMFT) is a benign fibro-osseous lesion that is characterized by mixture of histologic elements including lipoma, fibroxanthoma, myxoma, ischemic ossification, and fibrous dysplasia (FD)-like features. These tissue components are seen in the original reports of FD; however, the relationship between LSMFT and FD is not clear. Point mutation of the alpha subunit of G protein (Gs alpha), which increases cyclic adenosine monophosphate formation, has been recognized as the cause of McCune-Albright syndrome as well as polyostotic and monostotic FD of bone. Gs alpha mutation at the Arg201 codon in 2 patients of LSMFT was demonstrated in the present study. Although direct sequencing analysis using the fresh-frozen materials could not detect the mutation, the polymerase chain reaction fragmentation length polymorphism (PCR-RFLP) disclosed the missense point mutation Gs alpha at the Arg201 codon in 2 cases involving LSMFT. This result strongly suggests that a subset of LSMFT is a variant form of FD.

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

STUDY DESIGN: Case report. The authors documented a patient presenting with multiple cellular schwannomas along the sciatic nerve. The patient had no stigmas of neurofibromatosis. Thirteen tumors were resected via both anterior and posterior approaches. OBJECTIVES: To point out that presacral cellular schwannoma can display multiple occurrences. SUMMARY OF BACKGROUND DATA: Cellular schwannoma, which is a well-recognized variant of benign schwannoma, is often misdiagnosed as sarcoma. The tumor is usually a solitary lesion. MATERIALS AND METHODS: Medical history, physical findings, imaging features, and histologic findings were reviewed in a case of multiple cellular schwannomas in the presacral regions. RESULTS: A 39-year-old woman without any evidence of neurofibromatosis underwent removal of presacral multiple tumors along the sacral nerve root. Nineteen years after the surgery, the tumors recurred with multiple fashion. Thirteen tumors were resected via both anterior and posterior approaches. Immunohistochemical analysis of S-100 protein and Ki-67 were beneficial with respect to differentiation of cellular schwannoma from malignant peripheral nerve sheath tumor. CONCLUSIONS: The present case illustrated that multiple cellular schwannomas can develop in nonneurofibromatosis patients.

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記述言語：日本語   掲載種別：研究論文（学術雑誌）  

OBJECTIVE: To clarify the high-resolution CT(HRCT) findings of pulmonary atypical adenomatous hyperplasia (AAH) of 5 mm or less in diameter. MATERIALS AND METHODS: We evaluated the HRCT findings of 43 histopathologically confirmed AAH of 5 mm or less in diameter in 7 patients who underwent lobectomy for pulmonary adenocarcinoma. For comparison, we also examined the HRCT findings of 13 bronchioloalveolar carcinomas (BAC) of the same size from these patients. RESULTS: We identified 36 of 43 AAH and all 13 BAC on HRCT performed with multidetector-row CT. Thirty-five AAH and 11 BAC showed ground-glass opacity without any high-attenuation component. Margins of 20 AAH were well defined, and 16 were ill defined. In BAC, 11 lesions demonstrated well-defined margins, with only 2 showing ill-defined margins. CONCLUSION: Most AAH lesions of 5 mm or less in diameter are identified as ground-glass opacity on HRCT. Detection of minute ground-glass opacity is important in locating AAH on HRCT.

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：BRITISH MED JOURNAL PUBL GROUP  

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掲載種別：研究論文（学術雑誌）   出版者・発行元：Wiley  

DOI： 10.1046/j.1440-1827.2002.01357.x

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

PubMed

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Prominent osteoconductive activity and the biodegradable nature of beta tricalcium phosphate (beta-TCP) for bone grafts in animal experiments has been reported. A new type of beta-TCP has been manufactured at extraordinarily high purity and has been available as potent bone grafting substitute for clinical use. The histological features of grafted beta-TCP in human bone have been analyzed. A 33-year-old female with a bone tumor of the proximal femur underwent curettage and beta-TCP graft under the diagnosis of probable benign fibrous dysplasia. Four weeks later, the proximal femur, including the grafted beta-TCP was resected because of the final diagnosis of the curettaged materials was osteosarcoma. The resected specimen revealed abundant direct new bone apposition on beta-TCP. There was no cartilaginous tissue or enchondral ossification. Bone formation was more prominent in the periphery of the grafted area than in the center. There was a considerable number of osteoclast-like giant cells surrounding the beta-TCP. This case illustrated that highly purified beta-TCP had prominent osteoconductive activity and biodegradable nature in human bone.

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

Background: Clear cell chondrosarcoma is a rate bone tumor, which is sometimes misdiagnosed as a different bone neoplasm. Materials and Methods: The files of 6 patients with clear cell chondrosarcomas were reviewed. Histological slides, radiographic studies, and pre- and post-operative serum alkaline phosphatase (ALP) levels were evaluated. Molecular and histochemical analyses of ALP were documented in one case of clear cell chondrosarcoma. Results: Pre-operative serum ALP levels were elevated in 3 patients, and were normal in another 3 patients. After removal of the tumors, the enzyme levels decreased in all patients and returned to normal in 3 patients, who had pre-operative high ALP levels. Enzyme histochemical and molecular analyses demonstrated that the tumor produced ALP. Conclusion: Clear cell chondrosarcoma produces ALP, which can be used as a tumor marker in diagnosis and follow-up.

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