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Overview of natural history, pathology, molecular genetics and management of HNPCC (Lynch syndrome)
Author(s): Lynch HT, Smyrk T, Lynch JF
Source: INTERNATIONAL JOURNAL OF CANCER    Volume: 69    Issue: 1    Pages: 38-43    Published: FEB 20 1996  
Times Cited: 136     References: 52     
Abstract: Hereditary non-polyposis colorectal cancer (HNPCC) predisposes to cancers of the colon, endometrium and several other extra-colonic sites in the absence of premonitory physical stigmata (Muir-Torre syndrome excepted). Discovery of the several DNA mismatch repair genes (hMSH2, hMLHI, hPMS I, hPMS2) holds the potential for determining the cancer destiny of patients, theoretically in utero. pre-symptomatic DNA testing is now possible in patients from HNPCC families and will be clinically available once inexpensive and simple tests for these germ-line mutations have been effected. Genetic counseling will be mandatory, given the myriad socio-psychological, insurance, and potentially other personal issues which may impact this knowledge. New findings in the pathology of HNPCC, particularly an increased frequency of interval cancers and the likely accelerated rate of the adenoma to cancer sequence, indicate the need for more frequent colonoscopic surveillance with an option for prophylactic subtotal colectomy in germ-line-positive individuals. (C) 1996 Wiley-Liss, Inc.
Document Type: Proceedings Paper
Language: English
Reprint Address: Lynch, HT (reprint author), CREIGHTON UNIV, SCH MED, DEPT PREVENT MED, 2500 CALIF PLAZA, OMAHA, NE 68178 USA
Addresses:
1. CREIGHTON UNIV, DEPT PATHOL, OMAHA, NE 68178 USA
Publisher: WILEY-LISS, DIV JOHN WILEY & SONS INC 605 THIRD AVE, NEW YORK, NY 10158-0012
Subject Category: Oncology
IDS Number: TX092
ISSN: 0020-7136
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