Alveolar soft part sarcoma in children and adolescents: A report from the Soft-Tissue Sarcoma Italian Cooperative Group

Background: Alveolar soft part sarcoma (ASPS) is a rare malignant tumor and little is known about its clinical features and management. We report on a series of 19 pediatric patients managed over 20 years. Patients and methods: Primary conservative surgery was performed in all pat ients and was radical in nine, non-radical in three; seven patients underwe nt biopsy alone (3 unresectable tumors, 4 metastatic disease). In two cases radical surgery was performed after primary chemotherapy. Radiotherapy was delivered to 8 patients, chemotherapy to 15. Results: After a median follow-up of 74 months, the five-year survival was 80% for the whole series, 91% for patients with localized disease, 100% for patients with tumor less than or equal to5 cm, and 31% for those >5 cm; 16 of 19 patients were alive (12 of 12 with grossly-resected tumor in first c ontinuous remission). Chemotherapy achieved two partial remission among sev en evaluable patients. Conclusions: Pediatric ASPS has a more favorable prognosis than its adult c ounterpart. In this series, tumor size correlates with metastatic disease a t onset and is the major factor influencing survival. Surgery is the mainst ay of therapy. The effectiveness of adjuvant therapy remains to be establis hed, though radiotherapy may be advisable in cases of inadequate surgery.