Document Detail


Young age and a positive family history of colorectal cancer are complementary selection criteria for the identification of Lynch syndrome.
MedLine Citation:
PMID:  21273057     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Families at high risk for Lynch syndrome can effectively be recognised by microsatellite instability (MSI) testing. The aim of the present study is to compare the effectiveness of a MSI test for the identification of Lynch syndrome in patients selected by a pathologist mainly based on young age at diagnosis (MSI-testing-indicated-by-a-Pathologist; MIPA), with that of patients selected by a clinical geneticist mainly based on family history (MSI-testing-indicated-by-Family-History; MIFH). Patients with a Lynch syndrome associated tumour were selected using MIPA (n=362) or MIFH (n=887). Germline DNA mutation testing was performed in 171 out of 215 patients (80%) with a MSI positive tumour. MSI was tested positive in 20% of the MIPA-group group compared to 16% in the MIFH-group (P=0.291). In 91 of 171 patients with MSI positive tumours tested for germline mutations were identified as Lynch syndrome patients: 42% in the MIPA-group and 56% in the MIFH-group (P=0.066). Colorectal cancer (CRC) or endometrial cancer (EC) presenting at an age below 50years would have led to the diagnosis of Lynch syndrome in 89% of these families (CRC below 50years: 88% and EC below 50years: 12%). Families detected by MIPA were characterised more often by extracolonic Lynch syndrome associated malignancies, especially EC (P<0.001). Our results indicate that recognition of Lynch syndrome by CRC or EC below 50years is as effective as a positive family history. Families from patients selected by individual criteria more often harbour extracolonic Lynch syndrome associated malignancies.
Authors:
P Manders; L Spruijt; C M Kets; H W Willems; D Bodmer; K M Hebeda; I D Nagtegaal; J H J M van Krieken; M J L Ligtenberg; N Hoogerbrugge
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-1-25
Journal Detail:
Title:  European journal of cancer (Oxford, England : 1990)     Volume:  -     ISSN:  1879-0852     ISO Abbreviation:  -     Publication Date:  2011 Jan 
Date Detail:
Created Date:  2011-1-28     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9005373     Medline TA:  Eur J Cancer     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2010 Elsevier Ltd. All rights reserved.
Affiliation:
Department of Human Genetics, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
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