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图尔科特综合征:一例报告并文献复习

Turcot syndrome: report of a case and review of the literature.

作者信息

Jamjoom Z A, Sadiq S, Mofti A B, al-Mofleh I, Ajarim D

机构信息

Division of Neurosurgery, College of Medicine, King Saud University, Riyadh, Saudi Arabia.

出版信息

Int Surg. 1989 Jan-Mar;74(1):45-50.

PMID:2540108
Abstract

We report a case of Turcot's syndrome in a 20-year old man with multiple adenomatous polyps of the colon and glioblastoma multiforme. Detailed histopathological study of all 25 polyps removed from his colon confirmed the distinct morphological and numerical features of the colonic polyposis in Turcot's syndrome. Moreover, 45% of the total polyps and all polyps with a diameter exceeding 2 cm showed malignant transformation, indicating the precancerous nature of these polyps. These findings are discussed together with data obtained from a literature review of 32 histopathologically confirmed cases of Turcot's syndrome with reference to the possible heterogeneous nature of the syndrome at the present time. The current views on the relationship of Turcot's syndrome to other polyposis coli syndromes are presented.

摘要

我们报告一例20岁男性的Turcot综合征,该患者患有结肠多发腺瘤性息肉和多形性胶质母细胞瘤。对从其结肠切除的所有25个息肉进行的详细组织病理学研究证实了Turcot综合征中结肠息肉病独特的形态学和数量特征。此外,全部息肉的45%以及所有直径超过2 cm的息肉均发生了恶性转化,表明这些息肉具有癌前性质。结合对32例经组织病理学确诊的Turcot综合征病例的文献综述所获得的数据,对这些发现进行了讨论,同时参考了该综合征目前可能存在的异质性。本文还介绍了目前关于Turcot综合征与其他结肠息肉病综合征关系的观点。

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