Revised Bethesda Guidelines for Hereditary Nonpolyposis Colorectal Cancer (Lynch Syndrome) and Microsatellite Instability

Overview

Journal J Natl Cancer Inst

Publisher Oxford University Press

Specialty Oncology

Date 2004 Feb 19

PMID 14970275

Citations 1169

Authors

Asad Umar

C Richard Boland

Jonathan P Terdiman

Sapna Syngal

Albert de la Chapelle

Josef Ruschoff

Richard Fishel

Noralane M Lindor

Lawrence J Burgart

Richard Hamelin

Stanley R Hamilton

Robert A Hiatt

Jeremy Jass

Annika Lindblom

Henry T Lynch

Paivi Peltomaki

Scott D Ramsey

Miguel A Rodriguez-Bigas

Hans F A Vasen

Ernest T Hawk

J Carl Barrett

Andrew N Freedman

Sudhir Srivastava

Affiliations

Soon will be listed here.

Abstract

Hereditary nonpolyposis colorectal cancer (HNPCC), also known as Lynch syndrome, is a common autosomal dominant syndrome characterized by early age at onset, neoplastic lesions, and microsatellite instability (MSI). Because cancers with MSI account for approximately 15% of all colorectal cancers and because of the need for a better understanding of the clinical and histologic manifestations of HNPCC, the National Cancer Institute hosted an international workshop on HNPCC in 1996, which led to the development of the Bethesda Guidelines for the identification of individuals with HNPCC who should be tested for MSI. To consider revision and improvement of the Bethesda Guidelines, another HNPCC workshop was held at the National Cancer Institute in Bethesda, MD, in 2002. In this commentary, we summarize the Workshop presentations on HNPCC and MSI testing; present the issues relating to the performance, sensitivity, and specificity of the Bethesda Guidelines; outline the revised Bethesda Guidelines for identifying individuals at risk for HNPCC; and recommend criteria for MSI testing.

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