Abstract

Muir Torre Syndrome (MTS) is a rare autosomal dominant phenotypic variant of hereditary nonpolyposis colorectal carcinoma (HNPCC). It is characterized by the presence of at least one visceral malignancy and one sebaceous skin tumor. Patients have a strong family history of primary cancers and the Amsterdam
criteria or the Mayo Muir Torre Risk Score are frequently used to diagnose it. We present a case of a 58 years old who initially presented with a request to repair two incisional hernias following a procedure at another institution. Past family history was remarkable for strong history of colon, lung, and skin cancer in his father and 3 brothers. On CT, the patient was found to have a mass in the ascending colon and the prostate suggesting primary colon carcinoma and prostate adenocarcinoma. The patient was found to have a 5cm lesion on his left upper back, which was excised, biopsied and found to be sebaceous carcinoma. Given the constellation of sebaceous skin and visceral tumors, and after the pathological study of the malignancies a diagnosis of Muir Torre Syndrome was made. Muir Torre Syndrome is a rare phenotypic variant of HNPCC associated with a poor prognosis. We present an interesting case of Muir Torre Syndrome that was diagnosed due to a detailed medical history and physical exam. This case highlights the need for thorough family history, physical examination, and screening for suspected malignancies.