Academic Journals Database
Disseminating quality controlled scientific knowledge

Gynecological tumors in patients with Peutz-Jeghers syndrome (PJS)

ADD TO MY LIST
 
Author(s): Arisa Ueki | Iori Kisu | Kouji Banno | Megumi Yanokura | Kennta Masuda | Yusuke Kobayashi | Akira Hirasawa | Daisuke Aoki

Journal: Open Journal of Genetics
ISSN 2162-4453

Volume: 01;
Issue: 03;
Start page: 65;
Date: 2011;
Original page

Keywords: Gynecologic Tumor | Minimal Deviation Adenocarcinoma | Peutz-Jeghers Syndrome | Sex Cord Tumor | STK11/LKB1

ABSTRACT
Peutz-Jeghers syndrome (PJS) is an autosomal dominant disorder characterized by the development of hamartomatous polyposis in the gastrointestinal tract and melanin-pigmented macules on the skin mucosa. The responsible gene is a tumor suppressor, STK11/LKB1, on chromosome 19p13.3. PJS complicates with benign and malignant tumors in various organs. In gynecology, there has been a particular focus on complications of PJS with sex cord tumor with annular tubules (SCTAT) and minimal deviation adenocarcinoma (MDA), which are rare diseases. Approximately 36% of patients with SCTAT are complicated with PJS and these patients are characterized by multifocal, bilateral, small and benign lesions that develop into tumors with mucinous to serous ratios of 8:1. In addition, 10% of cases of MDA are complicated with PJS and mutation of STK11, the gene responsible for PJS, has a major effect on onset and prognosis. The disease concept of lobular endocervical glandular hyper-plasia (LEGH) has recently been proposed and LEGH is thought to be a potential premalignant lesion of MDA, however, the relationship between PJS and LEGH remains unclear. Several case reports of PJS patients complicated with gynecological tumors have been published and further studies are needed to determine the underlying causes

Tango Rapperswil
Tango Rapperswil

     Save time & money - Smart Internet Solutions