ALVEOLAR SOFT PART SARCOMA IN CHILDREN AND ADOLESCENTS - CLINICAL-FEATURES AND OUTCOME OF 11 PATIENTS

The clinical features and response to therapy of pediatric alveolar so ft part sarcoma, a rare soft tissue sarcoma of uncertain histogenesis, have not been previously described in detail in the literature. We re trospectively reviewed the clinical characteristics of all patients wi th alveolar soft part sarcoma who were seen at our institution over a 32-year period. We found 11 patients with the diagnosis of alveolar so ft part sarcoma. Their ages ranged from 2.8-16 years (median 9.8). Sta ging was determined using the Intergroup Rhabdomyosarcoma Study clinic al grouping system and the UICC TNM system. Accordingly, there were si x patients with grossly resected tumors (clinical groups I and it) and five with unresected or metastatic disease (clinical groups III and I V). Children with resected disease were more likely to have smaller no ninvasive tumors. The main feature predictive of survival was tumor re sectability, since chemotherapy in various combinations failed to prod uce significant tumor responses. Nine patients are disease-free with a median follow-up of 11.9 years. Surgical resection remains the mainst ay of therapy for pediatric alveolar soft part sarcoma. Since active c hemotherapy agents have not been identified, patients with unresected or metastatic disease may benefit from experimental agents. The surviv al rate of this cohort is superior to that seen in adults. (C) 1996 Wi ley-Liss, Inc.