Document Detail


Muir-Torre syndrome: Diagnostic and screening guidelines.
MedLine Citation:
PMID:  17034469     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
A 65-year-old man presented with a history of multiple skin coloured papules on his face that were asymptomatic. He had an adenocarcinoma resected from his proximal colon 12 years prior to presentation as well as a family history of colon cancer on the maternal side. Diagnostic biopsies showed the lesions to be sebaceous adenomas and epitheliomas and the diagnosis of Muir-Torre syndrome was made. The sebaceous tumour tissue showed microsatellite instability and immunohistochemical staining indicated diminished expression in the DNA mismatch-repair protein complex MSH2/MSH6. Genetic analysis showed a germline mutation in the MSH2 gene confirming the diagnosis of Muir-Torre syndrome. The patient and his first-degree relatives have been referred for genetic counselling and screening. We review the diagnostic criteria in this syndrome and review the recommended screening guidelines.
Authors:
Brad Jones; Carol Oh; Elisabeth Mangold; Conleth A Egan
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  The Australasian journal of dermatology     Volume:  47     ISSN:  0004-8380     ISO Abbreviation:  Australas. J. Dermatol.     Publication Date:  2006 Nov 
Date Detail:
Created Date:  2006-10-12     Completed Date:  2007-01-09     Revised Date:  2009-11-19    
Medline Journal Info:
Nlm Unique ID:  0135232     Medline TA:  Australas J Dermatol     Country:  Australia    
Other Details:
Languages:  eng     Pagination:  266-9     Citation Subset:  IM    
Affiliation:
Department of Dermatology, Our Lady of Lourdes Hospital, Drogheda, Ireland. bradjones@ausdoctors.net
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MeSH Terms
Descriptor/Qualifier:
Aged
Colorectal Neoplasms, Hereditary Nonpolyposis / diagnosis*,  genetics*,  pathology
DNA Mismatch Repair
Diagnosis, Differential
Face / pathology
Genetic Predisposition to Disease*
Genetic Testing / standards
Humans
Male
Practice Guidelines as Topic
Sebaceous Gland Neoplasms / diagnosis*,  genetics*,  pathology

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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