Peutz-Jeghers' syndrome with malignant development in a hamartomatous polyp: Report of one case and review of the literature
Peutz-Jeghers' syndrome with malignant development in a hamartomatous polyp: Report of one case and review of the literature
复制标题
DOI：
10.1016/j.gcb.2008.01.009
复制
发表时间：
2008-03-01
影响因子：
--
通讯作者：
Mzabi-Regaya, S.
中科院分区：
文献类型：
--
作者：
Bouraoui, S.;Azouz, H.;Mzabi-Regaya, S.
The malignant potential of hamartomatous polyps in. Peutz-Jeghers' (PPJ) syndrome has been debated. Although it is a very rare event, these polyps can become malignant, as demonstrated by this report. One case of colonic adenocarcinoma associated with Peutz-Jeghers' syndrome is described in a 62-year-old woman. The patient had colonic carcinoma which developed in a hamartomatous polyp. The malignant development of this colonic hamartomatous polyp arising in Peutz-Jeghers' syndrome was pathologically confirmed at surgery. This case also shows a sequence of hamartoma-dysplasia-carcinoma in a hamartomatous polyp without adenomatous changes. This suggests that hamartomatous polyps in Peutz-Jeghers' syndrome may develop into adenocarcinoma and may be a precursor of gastrointestinal carcinomas. STK 11 is a tumor suppressor gene regulating the development of hamartomas, and this somatic mutation promotes gastrointestinal cancer at later stages in Peutz-Jeghers' syndrome. (C) 2008 Elsevier Masson SAS. Tous droits reserves.
