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家族性腺瘤性息肉病相关的上消化道腺瘤中并存的类癌肿瘤。

Coexisting carcinoid tumors in familial adenomatous polyposis-associated upper intestinal adenomas.

作者信息

July L V, Northcott K A, Yoshida E M, Carr D M, Owen D A

机构信息

Department of Surgery, University of British Columbia, Vancouver, Canada.

出版信息

Am J Gastroenterol. 1999 Apr;94(4):1091-4. doi: 10.1111/j.1572-0241.1999.01020.x.

Abstract

Upper gastrointestinal polyps and extraintestinal tumors are well recognized in association with familial adenomatous polyposis (FAP). Although carcinoid tumors have been reported in association with sporadic colonic neoplasms and ulcerative colitis, to date, carcinoids have not been reported in association with FAP. We report a patient with FAP who has recurrent carcinoid tumors located at the bases of duodenal adenomas. The genetic basis of carcinoid neoplasms is still uncertain. This report may represent the clinical effect of the APC gene mutation on the enterochromaffin cell line manifesting as recurrent carcinoid tumors in physical association with intestinal adenomas. Future genetic analysis and epidemiological studies may be of value in determining whether a true association exists.

摘要

上消化道息肉和肠外肿瘤与家族性腺瘤性息肉病(FAP)相关已得到充分认识。尽管类癌肿瘤已被报道与散发性结肠肿瘤和溃疡性结肠炎有关,但迄今为止,尚未有类癌肿瘤与FAP相关的报道。我们报告一例FAP患者,其十二指肠腺瘤底部反复出现类癌肿瘤。类癌肿瘤的遗传基础仍不确定。本报告可能代表了APC基因突变对肠嗜铬细胞系的临床影响,表现为与肠道腺瘤实际相关的复发性类癌肿瘤。未来的基因分析和流行病学研究可能对确定是否存在真正的关联有价值。

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