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Annals of Oncology 11:1445-1449, 2000
© 2000 European Society for Medical Oncology

research-article

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

M. Casanova1,, A. Ferrari1, G. Bisogno2, G. Cecchetto2, E. Basso3, B. De Bernardi4, P. Indolfi5, F. Fossati Bellani1 and M. Carli2

1Pediatric Oncology Unit, Istituto Nazionale Tumori Milan
2Division of Hematology-Oncology, Department of Pediatrics, University of Padua Padua
3Department of Pediatrics, University of Turin Turin
4Department of Hematology-Oncolog Giannina Gaslini Children's Hospital Genoa
5Pediatric Oncology Service, University of Naples Federico II Naples, Italy

Correspondence to: M. Casanova, MD Pediatric Oncology Unit Istituto Nazionale Tumori Via G. Venezian, 1 20133 Milano Italy E-mail: casanova{at}istitutotumori.mi.it

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 patients and was radical in nine, non-radical in three; seven patients underwent 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 ≤5 cm, and 31% for those >5 cm; 16 of 19 patients were alive (12 of 12 with grossly-resected tumor in first continuous remission). Chemotherapy achieved two partial remission among seven evaluable patients.

CONCLUSIONS: Pediatric ASPS has a more favorable prognosis than its adult counterpart. In this series, tumor size correlates with metastatic disease at onset and is the major factor influencing survival. Surgery is the mainstay of therapy. The effectiveness of adjuvant therapy remains to be established, though radiotherapy may be advisable in cases of inadequate surgery.

alveolar soft part sarcoma, pediatric tumors, soft tissue sarcoma


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