Clinical Follow-up and Presence of Visceral Tumors in 12 Patients With Sebaceous Gland Tumors (Lynch Syndrome & Muir-Torre Syndrome & clear cell)

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Reports of MTS in families with hereditary nonpolyposis colorectal cancer (HNPCC), also known as Lynch
syndrome,8 appeared for the first time in the 1980s. It was subsequently discovered that patients with MTS and HNPCC had the same genetic defect involving proteins responsible for DNA mismatch repair.9,10 It is now believed that MTS is a clinical form of HNPCC.11

Visceral malignancies in HNPCC typically affect the colon, but they can also be found in the endometrium, the ovary, the stomach, the small intestine, the ureter, the renal pelvis, and the brain.12 Other features of MTS include the presence of multiple tumors, early-age onset of tumors, and, in some cases, improved prognosis when tumors appear spontaneously.11

Nonetheless, it should be borne in mind that breast cancer and indeed other cancers described
in patients with MTS have not been definitively associated with HPNCC,25 meaning that their existence may, on occasions, be purely coincidental. Another group of authors proposed performing a computed tomography scan of the abdomen and pelvis every 2 to 5 years19 because 35% of abdominal tumors in MTS occur at sites other than the colon.27

Dermatologists play a key diagnostic role in MTS. Our findings suggest that some dermatologists underestimate the potential severity of sebaceous tumors, disregard the importance of family history of cancer, and fail to order additional studies to exclude visceral malignancies.