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Surveillance and Management of Hereditary Nonpolyposis Colorectal Cancer (HNPCC) Motohisa Takami 1 , Masaharu Kimura 1 1Department of Surgery, Toyonaka Municipal Hospital Keyword: HNPCC , 癌多発家系 , サーベイランス , マネージメント pp.851-862
Published Date 1996/6/25
DOI https://doi.org/10.11477/mf.1403104168
  • Abstract
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 Hereditary nonpolyposis colorectal cancer (HNPCC) is an autosomal dominant inherited disease characterized by the development of colorectal carcinoma at an early age, a predominance of tumors in the right-sided colon, and a high incidence of synchronous and metachronous colorectal cancers. HNPCC can be divided into two categories: (1) hereditary site-specific colorectal cancer, or Lynch syndrome Ⅰ which is characterized by the development of colorectal cancers only, and (2) Lynch syndrome Ⅱ which is defined as a combination of colorectal cancer and extracolonic cancers.

 In this article, we discuss strategy for the surveillance and management of HNPCC based on our experience. Regular colonoscopic examination is a very useful method for families with Lynch syndrome Ⅰ. As a costeffective screening for Lynch syndrome Ⅱ, both colonoscopic and gastroscopic examinations are recommended because carcinoma of the stomach is the most common extracolonic tumor in Japanese patients with this syndrome.

 The recent discovery of mismatch-repair genes responsible for the development of colorectal and other cancers in HNPCC may facilitate a presymptomatic diagnosis of this genetic entity. The most important lesson for clinicians is the importance of obtaining detailed family history of all cancer patients and the need for life long follow-up for all HNPCC patients and, perhaps, for all young cancer patients.


Copyright © 1996, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1219 印刷版ISSN 0536-2180 医学書院

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