Syndrome in question

Abstract

Muir-Torre syndrome is a rare genodermatosis characterized by the occurrence of at least one sebaceous tumor associated with visceral neoplasia, but with no predisposing factors. The sebaceous neoplasm may appear before, during or after the diagnosis of colorectal cancer. As it is regarded as a subtype of nonpolyposis hereditary colorectal cancer, it is important to evaluate the patient's first-degree relatives. The clinical course of the neoplasm is usually more indolent and the syndrome has a good prognosis. We report the case of a patient who, after a ten-year diagnosis of colorectal cancer, presented with multiple sebaceous neoplasms.

A possible association between mycosis fungoides and Muir-Torre syndrome: Two disorders with microsatellite instability

“…Despite the extreme rarity of both MF and MTS, our patient represents the third reported case of MF in a patient with MTS. 12 , 13 Evidence of MSI-H and MLH1 promoter hypermethylation in both MF and MTS suggests a subset of MF cases might share the same molecular pathogenesis as cases of sebaceous neoplasms or gastrointestinal malignancies in MTS. A disease association notwithstanding, MLH1 and MSH2 might have clinical relevance in MF.…”