Cystic sebaceous carcinoma: is it a constant pathognomic marker for Muir-Torre syndrome?

Sebaceous carcinoma (SC) is a rare and aggressive cutaneous neoplasm. It may arise in ocular or extraocular sites. Approximately 25% of all reported cases of SC are extraocular. Cystic presentation of sebaceous neoplasm is rare. So far, cystic sebaceous neoplasia (CSN) has been reported only in association with Muir-Torre syndrome (MTS). Furthermore, CSN has recently been characterized as a marker lesion of MTS. We report a case of CSN of the nose that was not associated with MTS. Mohs micrographic surgery was performed with no recurrence for 2 years. Patients with MTS need long-term follow-up to detect possible future presentation of MTS.