Muir-Torre, syndrome
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[MIM 153 320]
(Lynch II syndrome)
Rare. Autosomal dominant transmission of a mutation of either the MLH1 gene on 3q21.3 or the MSH2 gene on 2p22-p21.
These genes repair mismatched DNA following a replication error.
Association of:
- skin tumours: sebaceous gland (adenoma), keratoacanthomas, especially at the level of the face or trunk
- one or more visceral tumours: digestive tract (60 %), urogenital (20 %) or mammary
It is one of the causes of colorectal cancer without polyposis (cfr Lynch type 2 syndrome)
Anesthetic implications:
frequent endoscopies, colorectal surgery of the adolescent or young adult.
References:
Updated: December 2018