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.