M Aparicio-Domínguez, S Berenguer-Ruiz, L Turrión-Merino, I Alcaraz León, M Menéndez-Sánchez, C F Caballero-Linares, R García Castro, S Medina-Montalvo, P Muñoz-Hernández, P Rodríguez-Jiménez
PCL generally has a favorable prognosis, although the high proportion of late recurrences reinforces the need for close and prolonged clinical follow-up.
BACKGROUND: Primary cutaneous leiomyosarcoma (PCL) is a rare tumor of smooth muscle origin.
METHODS: We analyzed 39 cases of PCL diagnosed between 1998 and 2023 at 5 hospitals in Madrid, Spain.
RESULTS: The median age was 79 years, with a predominance of men (68%) and preferential involvement of the scalp (36%). Overall, 92% of tumors were T1 (<5 cm), and 62% showed predominantly dermal involvement on histopathology. The local recurrence rate was 18%, regardless of depth of invasion. Local recurrence was less frequent after Mohs surgery (12%) than after conventional surgery (28% with 1-cm margins and 16% with margins >1 cm). Three patients (8%) developed metastases, with only 1 tumor-related death. The 5-year disease-free survival rate was 80%.
CONCLUSIONS: PCL generally has a favorable prognosis, although the high proportion of late recurrences reinforces the need for close and prolonged clinical follow-up.