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胃孤立性丛状神经纤维瘤:一例报告

Solitary plexiform neurofibroma of the stomach: a case report.

作者信息

Shi Lei, Liu Fu-Jian, Jia Qiu-Hong, Guan Hang, Lu Zhao-Jiong

机构信息

Lei Shi, Fu-Jian Liu, Qiu-Hong Jia, Hang Guan, Zhao-Jiong Lu, Department of Gastroenterology, the Eighth Affiliated Hospital of Guangxi Medical University, Guigang 537100, Guangxi Zhuang Autonomous Region, China.

出版信息

World J Gastroenterol. 2014 May 7;20(17):5153-6. doi: 10.3748/wjg.v20.i17.5153.

Abstract

Plexiform neurofibroma (PN) of the digestive tract is very rare and usually part of the generalized syndrome of neurofibromatosis type 1 (von Recklinghausen disease). Solitary PN of the stomach is extremely rare and has not been reported in the literatures. Here we present a case of solitary PN of the stomach, which was not associated with von Recklinghausen disease. A 38-year-old male presented abdominal pain and distention for 7 d. The patient underwent endoscopy of the upper gastrointestinal tract, which revealed a 3.5 cm protruding and cauliflower-shaped mass with a shallow 1 cm central ulcer in the greater curvature of the stomach. The lesion was removed by laparoscopic surgery. Histological examination demonstrated characteristic histological findings of spindle-shaped cells. Immunohistochemical analysis showed that the tumor cells were positive for S-100 protein, but negative for CD34, KI-67, CD117, and actin. Based on histological findings, gastrointestinal stromal tumor could be excluded, and thus the case was confirmed as PN. We described the clinical features, physical examination, endoscopic findings, and histopathological examination of this case.

摘要

消化道丛状神经纤维瘤(PN)非常罕见,通常是1型神经纤维瘤病(冯雷克林霍增氏病)综合征的一部分。胃孤立性PN极为罕见,文献中尚未见报道。在此,我们报告一例胃孤立性PN,其与冯雷克林霍增氏病无关。一名38岁男性出现腹痛和腹胀7天。患者接受了上消化道内镜检查,结果显示胃大弯处有一个3.5厘米突出的菜花状肿物,中央有一个1厘米的浅表溃疡。该病变通过腹腔镜手术切除。组织学检查显示出梭形细胞的特征性组织学表现。免疫组织化学分析表明,肿瘤细胞S-100蛋白呈阳性,但CD34、KI-67、CD117和肌动蛋白呈阴性。根据组织学表现,可排除胃肠道间质瘤,因此该病例确诊为PN。我们描述了该病例的临床特征、体格检查、内镜检查结果和组织病理学检查情况。

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