Muir-Torre, syndrome

[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