Leiomyosarcoma in childhood and adolescence.

Abstract

Few series of leiomyosarcoma in patients < 21 years of age have been reported. We reviewed our institutional experience with this neoplasm to learn disease characteristics, patterns of relapse, and outcome. The records of 21 patients with leiomyosarcoma admitted to our institution were reviewed retrospectively; 18 of these were diagnosed after 1970. Overall survival was estimated using the Kaplan-Meier method. Ninety-five percent (20 of 21) were initially treated with a wide local excision that was complete with a negative microscopic margin in 10 (48%). There also was a strong correlation between grade and surgical margins. High-grade tumors were associated with a lower rate of complete resection. The majority underwent additional therapy. Radiation was used to treat both initial and recurrent disease in nine patients, with four of these undergoing brachytherapy. Thirteen patients were treated with adjuvant chemotherapy, most commonly doxorubicin (seven patients) and cisplatin (six patients). The median length of survival was 9.3 years, and there were nine disease-related deaths (43%). Of interest was the progressive decrease in survival with time. The 5-year overall survival rate was 79%; the 10-year rate was 49%. Three patients died of progressive disease > 10 years after initial diagnosis. We conclude that leiomyosarcomas arising in childhood and adolescence are associated with a good initial chance of survival that decreases progressively over time. Known prognostic factors from larger adult series are consistent with the present data, but they are not provable because of the small number of patients. In particular, the grade was correlated with surgical margins.