Muir-Torre Syndrome: A Case for Surveillance of the Ampulla of Vater

Abstract

A 64-year-old man with Muir-Torre syndrome (MTS), a condition characterized by the association of multiple sebaceous tumours and kerato-acanthomas with internal malignancies, developed a carcinoma of the ampulla of Vater. This is the 2nd reported ampullary carcinoma in 205 reported cases of MTS, suggesting a higher prevalence in MTS patients than in the general population. As an ampullary carcinoma is potentially amenable to surgical resection, particularly when detected early, patients with MTS should undergo regular endoscopic surveillance of the ampulla of Vater.