Cutaneous Leiomyosarcoma of the Head and Neck: Appreciation of a More Indolent Biological Behavior and Implications for Its Management

BACKGROUND: Leiomysosarcoma (LMS) is generally associated with high metastatic potential and poor survival. Cutaneous LMS (cLMS) has been considered a different entity with more indolent biological behavior. METHODS: A narrative review was performed to assess the biological behavior of cLMS, with special attention for the head and neck location. RESULTS: cLMS is located in the head and neck in 21%-33%. The local recurrence rate is 2%-18%, while only 3% develops metastatic disease at 10 years. The most important risk factor of recurrent disease is positive/close surgical margins, with a trend for increased risk at the head and neck. The vast majority of recurrences are local and appear during the first few years. No data support adjuvant treatment. The 5-year disease-specific survival is 96%-100%. CONCLUSIONS: cLMS exhibits a moderate risk of local recurrence, low metastatic potential, and excellent disease-specific survival. Surveillance may be focused on physical examination and limited in duration.