Transitional cell carcinoma in the Muir-Torre syndrome

The association of multiple sebaceous neoplasms and visceral malignancies is known as the Muir-Torre syndrome. We describe a patient who had papillary transitional cell carcinoma of the right renal pelvis resected in 1981 and papillary transitional cell carcinoma of the left renal pelvis removed surgically in 1983. Recurrent transitional cell carcinoma necessitated further resection of tumor in the right renal pelvis in 1983, right nephrectomy and partial ureterectomy in 1984, and removal of the right ureteral stump in 1985. History included multiple seborrheic keratoses, a keratoacanthoma and 4 sebaceous carcinomas between 1968 and 1985. In 1971 partial colectomy and jejunectomy were performed for stage D (Duke classification) adenocarcinoma of the transverse colon. Despite this the patient remains alive and well with no evidence of residual or metastatic disease. The Muir-Torre syndrome is a subtype of the cancer family syndrome, with gastrointestinal tract tumors being the most frequent visceral malignancies. Urinary tract carcinomas are present in just more than 10 per cent of the cases. This entity is discussed and the literature is reviewed with emphasis on the urological associations.