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一例表现为腹膜播散的原发性胃肠道外间质瘤

[A case of primary extragastrointestinal stromal tumor presenting as peritoneal dissemination].

作者信息

Yang Hong Jun, Kim Tae Ho, Park Min Kyoung, Lim Chang Hoon, Lee Kee Hyun, Kim Chang Whan, Han Sok Won, Kim Jean A

机构信息

Department of Internal Medicine, The Catholic University of Korea College of Medicine, Seoul, Korea.

出版信息

Korean J Gastroenterol. 2010 Nov;56(5):319-23. doi: 10.4166/kjg.2010.56.5.319.

Abstract

Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor of the gastrointestinal tract, but also occurs at a lower frequency in extra-gastrointestinal regions such as omentum, mesentery, retroperitoneum and undefined abdominal sites. This tumor is called extragastrointestinal stromal tumor (EGIST). EGIST is mostly diagnosed as a cystic mass, but rarely occurs as a disseminated abdominal tumor. We experienced a 70-year-old man with primary EGIST presenting as peritoneal dissemination. Abdominal CT showed diffuse peritoneal thickening with a large amount of ascites, but no definite mass lesion. Laparoscopic biopsy was performed and histologic findings showed tumor composed of epithelioid cells. In the results of immunohistochemical stains, the tumor showed positive reactivity with CD117 (c-kit), CD34, vimentin and actin, but negative reactivity with desmin and S-100 protein. On account of unresectability and histologic parameters of malignant behavior, he was started on imatinib.

摘要

胃肠道间质瘤(GIST)是胃肠道最常见的间叶性肿瘤,但也较少发生于胃肠道外区域,如大网膜、肠系膜、腹膜后及不明腹部部位。这种肿瘤被称为胃肠道外间质瘤(EGIST)。EGIST大多被诊断为囊性肿块,但很少以弥漫性腹部肿瘤形式出现。我们遇到一名70岁男性,原发性EGIST表现为腹膜播散。腹部CT显示弥漫性腹膜增厚伴大量腹水,但无明确肿块病变。进行了腹腔镜活检,组织学检查结果显示肿瘤由上皮样细胞组成。免疫组化染色结果显示,肿瘤对CD117(c-kit)、CD34、波形蛋白和肌动蛋白呈阳性反应,但对结蛋白和S-100蛋白呈阴性反应。由于无法切除且存在恶性行为的组织学参数,他开始接受伊马替尼治疗。

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