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一名无家族性腺瘤性息肉病的年轻女孩患肠系膜纤维瘤病。

Mesenteric fibromatosis in a young girl without familial adenomatous polyposis.

作者信息

Huerta Sergio, Heubner David R, Marcus Daniel R

机构信息

Department of Surgery, University of California, Irvine, Orange, CA 92868, USA.

出版信息

J Pediatr Surg. 2005 May;40(5):e33-6. doi: 10.1016/j.jpedsurg.2005.02.017.

Abstract

Mesenteric fibromatosis (MF) is a rare, benign tumor commonly associated with Gardner's syndrome. The signs and symptoms in patients with MF are insidious. Patients may present with abdominal pain or discomfort when the tumors reach large sizes, which is typical at the time of diagnosis. Differentiating MF from other neoplasms such as gastrointestinal stromal tumors may present a diagnostic dilemma, especially in patients without any history of familial adenomatous polyposis. In the present report, we discuss a young girl who presented with MF. A pertinent review of the literature is also presented. This case is peculiar in that MF presented at a young age in a patient without history of familial adenomatous polyposis and occurred in the omentum with local invasion to the stomach.

摘要

肠系膜纤维瘤病(MF)是一种罕见的良性肿瘤,通常与加德纳综合征相关。MF患者的体征和症状较为隐匿。当肿瘤长得很大时,患者可能会出现腹痛或不适,这在诊断时很常见。将MF与其他肿瘤如胃肠道间质瘤区分开来可能会带来诊断难题,尤其是在没有家族性腺瘤性息肉病病史的患者中。在本报告中,我们讨论了一名患有MF的年轻女孩。同时还对相关文献进行了综述。该病例的特殊之处在于,MF在一名没有家族性腺瘤性息肉病病史的年轻患者中出现,且发生在大网膜并局部侵犯胃。

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