回腸原発淡明細胞肉腫の1例
症例は41歳の女性で，発熱と血便，貧血を主訴に来院し，回腸腫瘍に対して回腸部分切除およびS状結腸切除を施行された．組織学的には，比較的小型で核胞体比が高く，淡明な胞体を持つ円形の異型細胞の敷石状増生を認めた．免疫組織学的に腫瘍細胞はS-100部分的に陽性，さらにSOX10がびまん性に陽性で，FISH法でEWSR1遺伝子のsplit signalを検出し，回腸原発の淡明細胞肉腫（clear cell sarcoma）と診断された．術後，doxorubicin（ADR）+ifosfamide（IFM）による化学療法を合計5コース行い，術後2年4か月を経過して明らかな再発は認めていない．極めてまれな...
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| Published in | 日本消化器外科学会雑誌 Vol. 49; no. 1; pp. 29 - 35 |
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| Main Authors | , , , , , , , , , |
| Format | Journal Article |
| Language | Japanese |
| Published |
一般社団法人 日本消化器外科学会
01.01.2016
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| Subjects | |
| Online Access | Get full text |
| ISSN | 0386-9768 1348-9372 |
| DOI | 10.5833/jjgs.2014.0247 |
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| Abstract | 症例は41歳の女性で，発熱と血便，貧血を主訴に来院し，回腸腫瘍に対して回腸部分切除およびS状結腸切除を施行された．組織学的には，比較的小型で核胞体比が高く，淡明な胞体を持つ円形の異型細胞の敷石状増生を認めた．免疫組織学的に腫瘍細胞はS-100部分的に陽性，さらにSOX10がびまん性に陽性で，FISH法でEWSR1遺伝子のsplit signalを検出し，回腸原発の淡明細胞肉腫（clear cell sarcoma）と診断された．術後，doxorubicin（ADR）+ifosfamide（IFM）による化学療法を合計5コース行い，術後2年4か月を経過して明らかな再発は認めていない．極めてまれな消化管原発の淡明細胞肉腫を経験したので報告する． |
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| AbstractList | 症例は41歳の女性で，発熱と血便，貧血を主訴に来院し，回腸腫瘍に対して回腸部分切除およびS状結腸切除を施行された．組織学的には，比較的小型で核胞体比が高く，淡明な胞体を持つ円形の異型細胞の敷石状増生を認めた．免疫組織学的に腫瘍細胞はS-100部分的に陽性，さらにSOX10がびまん性に陽性で，FISH法でEWSR1遺伝子のsplit signalを検出し，回腸原発の淡明細胞肉腫（clear cell sarcoma）と診断された．術後，doxorubicin（ADR）+ifosfamide（IFM）による化学療法を合計5コース行い，術後2年4か月を経過して明らかな再発は認めていない．極めてまれな消化管原発の淡明細胞肉腫を経験したので報告する． |
| Author | 小寺澤, 康文 松浦, 正徒 京極, 高久 橋本, 公夫 安川, 大貴 住井, 敦彦 石原, 美佐 伊丹, 淳 吉田, 真也 宇山, 直樹 |
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| References | 8) Hisaoka M, Ishida T, Kuo TT, Matsuyama A, Imamura T, Nishida K, et al. Clear cell sarcoma of soft tissue: a clinicopathologic, immunohistochemical, and molecular analysis of 33 cases. Am J Surg Pathol. 2008 Mar;32(3):452–460. 9) Kawai A, Hosono A, Nakayama R, Matsumine A, Matsumoto S, Ueda T, et al. Clear cell sarcoma of tendons and aponeuroses: a study of 75 patients. Cancer. 2007 ;109(1):109–116. 4) Stockman DL, Miettinen M, Suster S, Spagnolo D, Dominguez-Malagon H, Hornick JL, et al. Malignant gastrointestinal neuroectodermal tumor: clinicopathologic, immunohistochemical, ultrastructural, and molecular analysis of 16 cases with a reappraisal of clear cell sarcoma-like tumors of the gastrointestinal tract. Am J Surg Pathol. 2012 Jun;36(6):857–868. 5) Zambrano E, Reyes-Mugica M, Franchi A, Rosai J. An osteoclast-rich tumor of the gastrointestinal tract with features resembling clear cell sarcoma of soft parts: reports of 6 cases of a GIST simulator. Int J Surg Pathol. 2003 Apr;11(2):75–81. 6) Nonaka D, Chiriboga L, Rubin BP. Sox10: a pan-schwannian and melanocytic marker. Am J Surg Pathol. 2008 Sep;32(9):1291–1298. 7) Suárez-Vilela D, Izquierdo FM, Tojo-Ramallo S, R Riera-Velasco J, Escobar-Stein J. Malignant gastrointestinal neuroectodermal tumor showing overlapped immunophenotype with synovial sarcoma: CD99 and SOX10 antibodies are useful in differential diagnosis. Am J Surg Pathol. 2012 Dec;36(12):1905–1908. 10) Jones RL, Constantinidou A, Thway K, Ashley S, Scurr M, Al-Muderis O, et al. Chemotherapy in clear cell sarcoma. Med Oncol. 2011 Sep;28(3):859–863. 1) Enzinger FM. Clear-cell sarcoma of tendons and aponeuroses. An analysis of 21 cases. Cancer. 1965 Sep;18:1163–1174. 2) Ekfors TO, Kujari H, Isomaki M. Clear cell sarcoma of tendons and aponeuroses (malignant melanoma of soft parts) in the duodenum: the first visceral case. Histopathology. 1993 Mar;22(3):255–259. 3) Lasithiotakis K, Protonotarios A, Lazarou V, Tzardi M, Chalkiadakis G. Clear cell sarcoma of the jejunum: a case report. World J Surg Oncol. 2013 Jan;11: 17. |
| References_xml | – reference: 3) Lasithiotakis K, Protonotarios A, Lazarou V, Tzardi M, Chalkiadakis G. Clear cell sarcoma of the jejunum: a case report. World J Surg Oncol. 2013 Jan;11: 17. – reference: 5) Zambrano E, Reyes-Mugica M, Franchi A, Rosai J. An osteoclast-rich tumor of the gastrointestinal tract with features resembling clear cell sarcoma of soft parts: reports of 6 cases of a GIST simulator. Int J Surg Pathol. 2003 Apr;11(2):75–81. – reference: 9) Kawai A, Hosono A, Nakayama R, Matsumine A, Matsumoto S, Ueda T, et al. Clear cell sarcoma of tendons and aponeuroses: a study of 75 patients. Cancer. 2007 ;109(1):109–116. – reference: 8) Hisaoka M, Ishida T, Kuo TT, Matsuyama A, Imamura T, Nishida K, et al. Clear cell sarcoma of soft tissue: a clinicopathologic, immunohistochemical, and molecular analysis of 33 cases. Am J Surg Pathol. 2008 Mar;32(3):452–460. – reference: 2) Ekfors TO, Kujari H, Isomaki M. Clear cell sarcoma of tendons and aponeuroses (malignant melanoma of soft parts) in the duodenum: the first visceral case. Histopathology. 1993 Mar;22(3):255–259. – reference: 6) Nonaka D, Chiriboga L, Rubin BP. Sox10: a pan-schwannian and melanocytic marker. Am J Surg Pathol. 2008 Sep;32(9):1291–1298. – reference: 10) Jones RL, Constantinidou A, Thway K, Ashley S, Scurr M, Al-Muderis O, et al. Chemotherapy in clear cell sarcoma. Med Oncol. 2011 Sep;28(3):859–863. – reference: 1) Enzinger FM. Clear-cell sarcoma of tendons and aponeuroses. An analysis of 21 cases. Cancer. 1965 Sep;18:1163–1174. – reference: 4) Stockman DL, Miettinen M, Suster S, Spagnolo D, Dominguez-Malagon H, Hornick JL, et al. Malignant gastrointestinal neuroectodermal tumor: clinicopathologic, immunohistochemical, ultrastructural, and molecular analysis of 16 cases with a reappraisal of clear cell sarcoma-like tumors of the gastrointestinal tract. Am J Surg Pathol. 2012 Jun;36(6):857–868. – reference: 7) Suárez-Vilela D, Izquierdo FM, Tojo-Ramallo S, R Riera-Velasco J, Escobar-Stein J. Malignant gastrointestinal neuroectodermal tumor showing overlapped immunophenotype with synovial sarcoma: CD99 and SOX10 antibodies are useful in differential diagnosis. Am J Surg Pathol. 2012 Dec;36(12):1905–1908. |
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| Title | 回腸原発淡明細胞肉腫の1例 |
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