小腸原発類上皮血管肉腫の1例

症例は75歳，男性．嘔気と嘔吐を主訴に近医を受診し，貧血がみられたため当院紹介となった．腹部骨盤造影CTで上部空腸の小腸腫瘍を認め，小腸内視鏡では表層粘膜が発赤乳頭状で頂部に粘膜欠損を伴う隆起性病変を認めた．経過中に腫瘍出血による急速な貧血進行を認めたため，緊急で腹腔鏡補助下小腸部分切除術を施行した．病理組織学的所見では，核分裂像の目立つ腫瘍細胞が上皮のようにシート状に配列し，一部で管腔様構造の形成を認めた．免疫組織化学染色ではCD31とvimentinが陽性であり，小腸原発の類上皮血管肉腫と診断した．術後補助化学療法は施行せず経過観察していたが，腹膜播種の急速増大，多発肺転移を認め，術後約4...

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Published in日本臨床外科学会雑誌 Vol. 78; no. 3; pp. 521 - 526
Main Authors 皆川, 昌広, 安藤, 拓也, 谷, 達夫, 長谷川, 潤, 薄田, 浩幸, 内藤, 哲也, 島影, 尚弘
Format Journal Article
LanguageJapanese
Published 日本臨床外科学会 2017
Subjects
小腸
類上皮血管肉腫
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ISSN1345-2843
1882-5133
DOI10.3919/jjsa.78.521

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Abstract 症例は75歳，男性．嘔気と嘔吐を主訴に近医を受診し，貧血がみられたため当院紹介となった．腹部骨盤造影CTで上部空腸の小腸腫瘍を認め，小腸内視鏡では表層粘膜が発赤乳頭状で頂部に粘膜欠損を伴う隆起性病変を認めた．経過中に腫瘍出血による急速な貧血進行を認めたため，緊急で腹腔鏡補助下小腸部分切除術を施行した．病理組織学的所見では，核分裂像の目立つ腫瘍細胞が上皮のようにシート状に配列し，一部で管腔様構造の形成を認めた．免疫組織化学染色ではCD31とvimentinが陽性であり，小腸原発の類上皮血管肉腫と診断した．術後補助化学療法は施行せず経過観察していたが，腹膜播種の急速増大，多発肺転移を認め，術後約4カ月目に死亡した． 類上皮血管肉腫は皮膚や軟部組織に好発する血管内皮細胞由来の悪性腫瘍であり，消化管を原発とする症例は極めて稀である．本症例は小腸を原発とする貴重な症例と考えられたため報告する．
AbstractList 症例は75歳，男性．嘔気と嘔吐を主訴に近医を受診し，貧血がみられたため当院紹介となった．腹部骨盤造影CTで上部空腸の小腸腫瘍を認め，小腸内視鏡では表層粘膜が発赤乳頭状で頂部に粘膜欠損を伴う隆起性病変を認めた．経過中に腫瘍出血による急速な貧血進行を認めたため，緊急で腹腔鏡補助下小腸部分切除術を施行した．病理組織学的所見では，核分裂像の目立つ腫瘍細胞が上皮のようにシート状に配列し，一部で管腔様構造の形成を認めた．免疫組織化学染色ではCD31とvimentinが陽性であり，小腸原発の類上皮血管肉腫と診断した．術後補助化学療法は施行せず経過観察していたが，腹膜播種の急速増大，多発肺転移を認め，術後約4カ月目に死亡した． 類上皮血管肉腫は皮膚や軟部組織に好発する血管内皮細胞由来の悪性腫瘍であり，消化管を原発とする症例は極めて稀である．本症例は小腸を原発とする貴重な症例と考えられたため報告する．
Author 内藤, 哲也
長谷川, 潤
薄田, 浩幸
谷, 達夫
安藤, 拓也
皆川, 昌広
島影, 尚弘
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References 1) Dolores NC, Marco FR, Javier LG, et al : Obscure gastrointestinal bleeding due to multifocal intestinal angiosarcoma. Int J Surg Case Rep 2015 ; 10 : 169-172
6) Delvaux V, Sciot R, Neuville B, et al : Multifocal epithelioid angiosarcoma of the small intestine. Virchows Arch 2000 ; 437 : 90-94
13) Chen M, Zhang W, Qu J, et al : Epithelioid angiosarcoma of the ilium : a case report. Int J Clin Exp Pathol 2014 ; 7 : 9099-9103
5) Al Ali J, Ko HH, Owen D, et al : Epithelioid angiosarcoma of the small bowel. Gastrointest Endosc 2006 ; 64 : 1018-1021
7) Leroy X, Saint-Blancard P, Boucher E, et al : Multifocal epithelioid angiosarcoma of the small intestine. Apropos of a case. Arch Anat Cytol Pathol 1997 ; 45 : 54-56
10) Watanabe K, Hoshi N, Suzuki T, et al : Epithelioid angiosarcoma of the intestinal tract with endothelin-1-like immunoreactivity. Virchows Arch A Pathol Anat Histopathol 1993 ; 423 : 309-314
14) Hanai J, Kimura K, Li T, et al : Malignant vascular tumor originating from pancreas : Epithelioid angiosarcoma. Arch Histopathol Different Diagn 2010 ; 17 : 40-43
15) Deléaval JP, Peter MY, Laurencet F, et al : Multicentric intestinal angiosarcoma. Report on a case. Ann Pathol 1991 ; 11 : 342-344
18) Wu J, Li X, Liu X : Epithelioid angiosarcoma : a clinicopathological study of 16 Chinese cases. Int J Clin Exp Pathol 2015 ; 8 : 3901-3909
4) Khalil MF, Thomas A, Aassad A, et al : Epithelioid Angiosarcoma of the Small Intestine After Occupational Exposure to Radiation and Polyvinyl Chloride : A case Report and Review of Literature. Sarcoma 2005 ; 9 : 161-164
3) Grewal JS, Daniel AR, Carson EJ, et al : Rapidly progressive metastatic multicentric epithelioid angiosarcoma of the small bowel : a case report and a review of literature. Int J Colorectal Dis 2008 ; 23 : 745-756
16) 堀木紀行，丸山正隆，藤田善幸他:胃，大腸に散発した悪性血管内皮腫の1例．Gastroenterol Endosc　1993;35:1334-1338
2) 池田　貯，唐原和秀，佐藤大亮他:直腸に原発した類上皮血管肉腫の1例．日消外会誌　2005;38:262-267
11) Kuroda N, Hamaguchi N, Inoue K, et al : Application of immunocytochemistry to the diagnosis of primary epithelioid angiosarcoma of the lung. Med Mol Morphol 2009 ; 42 : 250-253
8) Tanaka H, Yorita K, Takahashi N, et al : Primary pulmonary angiosarcoma : A case report. Pathol Int 2015 ; 65 : 554-557
9) Obeso Carillo GA, García Fontán EM, Cañizares Carretero MÁ, et al : Primary pulmonary angiosarcoma, an exceptional neoplasm with a poor prognosis : reports of two cases and review of the literature. Gen Thorac Cardiovasc Surg 2013 ; 61 : 643-647
12) Folpe AL, Chand EM, Goldblum JR, et al : Expression of Fli-1, a nuclear transcription factor, distinguishes vascular neoplasms for potential mimics. Am J Surg Pathol 2011 ; 25 : 1061-1066
17) Rossi S, Orvieto E, Furlanetto A, et al : Utility of the immunohistochemical detection of FLI-1 expression in round cell and vascular neoplasm using a monoclonal antibody. Mod Pathol 2004 ; 17 : 547-552
References_xml – reference: 3) Grewal JS, Daniel AR, Carson EJ, et al : Rapidly progressive metastatic multicentric epithelioid angiosarcoma of the small bowel : a case report and a review of literature. Int J Colorectal Dis 2008 ; 23 : 745-756
– reference: 16) 堀木紀行，丸山正隆，藤田善幸他:胃，大腸に散発した悪性血管内皮腫の1例．Gastroenterol Endosc　1993;35:1334-1338
– reference: 17) Rossi S, Orvieto E, Furlanetto A, et al : Utility of the immunohistochemical detection of FLI-1 expression in round cell and vascular neoplasm using a monoclonal antibody. Mod Pathol 2004 ; 17 : 547-552
– reference: 13) Chen M, Zhang W, Qu J, et al : Epithelioid angiosarcoma of the ilium : a case report. Int J Clin Exp Pathol 2014 ; 7 : 9099-9103
– reference: 10) Watanabe K, Hoshi N, Suzuki T, et al : Epithelioid angiosarcoma of the intestinal tract with endothelin-1-like immunoreactivity. Virchows Arch A Pathol Anat Histopathol 1993 ; 423 : 309-314
– reference: 8) Tanaka H, Yorita K, Takahashi N, et al : Primary pulmonary angiosarcoma : A case report. Pathol Int 2015 ; 65 : 554-557
– reference: 11) Kuroda N, Hamaguchi N, Inoue K, et al : Application of immunocytochemistry to the diagnosis of primary epithelioid angiosarcoma of the lung. Med Mol Morphol 2009 ; 42 : 250-253
– reference: 9) Obeso Carillo GA, García Fontán EM, Cañizares Carretero MÁ, et al : Primary pulmonary angiosarcoma, an exceptional neoplasm with a poor prognosis : reports of two cases and review of the literature. Gen Thorac Cardiovasc Surg 2013 ; 61 : 643-647
– reference: 18) Wu J, Li X, Liu X : Epithelioid angiosarcoma : a clinicopathological study of 16 Chinese cases. Int J Clin Exp Pathol 2015 ; 8 : 3901-3909
– reference: 1) Dolores NC, Marco FR, Javier LG, et al : Obscure gastrointestinal bleeding due to multifocal intestinal angiosarcoma. Int J Surg Case Rep 2015 ; 10 : 169-172
– reference: 12) Folpe AL, Chand EM, Goldblum JR, et al : Expression of Fli-1, a nuclear transcription factor, distinguishes vascular neoplasms for potential mimics. Am J Surg Pathol 2011 ; 25 : 1061-1066
– reference: 2) 池田　貯，唐原和秀，佐藤大亮他:直腸に原発した類上皮血管肉腫の1例．日消外会誌　2005;38:262-267
– reference: 14) Hanai J, Kimura K, Li T, et al : Malignant vascular tumor originating from pancreas : Epithelioid angiosarcoma. Arch Histopathol Different Diagn 2010 ; 17 : 40-43
– reference: 15) Deléaval JP, Peter MY, Laurencet F, et al : Multicentric intestinal angiosarcoma. Report on a case. Ann Pathol 1991 ; 11 : 342-344
– reference: 6) Delvaux V, Sciot R, Neuville B, et al : Multifocal epithelioid angiosarcoma of the small intestine. Virchows Arch 2000 ; 437 : 90-94
– reference: 7) Leroy X, Saint-Blancard P, Boucher E, et al : Multifocal epithelioid angiosarcoma of the small intestine. Apropos of a case. Arch Anat Cytol Pathol 1997 ; 45 : 54-56
– reference: 4) Khalil MF, Thomas A, Aassad A, et al : Epithelioid Angiosarcoma of the Small Intestine After Occupational Exposure to Radiation and Polyvinyl Chloride : A case Report and Review of Literature. Sarcoma 2005 ; 9 : 161-164
– reference: 5) Al Ali J, Ko HH, Owen D, et al : Epithelioid angiosarcoma of the small bowel. Gastrointest Endosc 2006 ; 64 : 1018-1021
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Snippet 症例は75歳，男性．嘔気と嘔吐を主訴に近医を受診し，貧血がみられたため当院紹介となった．腹部骨盤造影CTで上部空腸の小腸腫瘍を認め，小腸内視鏡では表層粘膜が発赤乳...
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SubjectTerms 小腸
類上皮血管肉腫
Title 小腸原発類上皮血管肉腫の1例
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