c-KIT positive Gastrointestinal Stromal Tumor presenting with acute bleeding in a patient with neurofibromatosis type 1: a case report.

International seminars in surgical oncology : ISSO Pub Date : 2009-10-23 DOI:10.1186/1477-7800-6-17

Esam Aboutaleb, Manish Kothari, Osama Damrah, Roben Canelo

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引用次数: 7

Abstract

Background: Gastrointestinal stromal tumours are rare (GIST). However, the incidence of GIST among neurofibromatosis type 1 (NF-1) patients is approximately 3.9-25%. GIST can present clinically in different ways such as abdominal pain, gastrointestinal bleeding and obstruction.

Case report: We present 51 year female patient admitted with Background of neurofibromatosis type 1 admitted with melena. OGD has been done and showed duodenitis with large volume fresh blood in distal duodenum but no obvious bleeding point. Exploratory laparotomy revealed smooth nodular masses on the serosal surface of jejunum. Small bowel resection and side-to-side anastomosis were performed. Histopathoogical examination revealed small bowel gasrointestinal stromal tumour with low risk malignant potential.

Conclusion: The incidence of GIST among neurofibromatosis type 1 (NF-1) patients is not uncommon and we should pay attention to gastrointestinal manifestation in such patients.

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1型神经纤维瘤患者c-KIT阳性胃肠道间质瘤伴急性出血1例报告。

背景:胃肠道间质瘤是罕见的。然而，GIST在1型神经纤维瘤病(NF-1)患者中的发病率约为3.9-25%。胃肠道间质瘤在临床上可表现为腹痛、胃肠道出血、梗阻等多种表现。病例报告:我们报告了51岁的女性患者，其背景为1型神经纤维瘤病，并伴有黑黑。OGD显示十二指肠炎，十二指肠远端有大量新鲜血液，但无明显出血点。剖腹探查发现空肠浆膜表面光滑结节状肿块。行小肠切除术及侧侧吻合。组织病理检查示小肠、胃肠道间质瘤，有低危恶性潜能。结论:胃肠道间质瘤病在1型神经纤维瘤病(NF-1)患者中发病率并不少见，应重视胃肠道表现。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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International seminars in surgical oncology : ISSO

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