Alveolar Soft Part Sarcoma: Clinical Experience in VGHTPE

Abstract

Background: Alveolar soft part sarcoma (ASPS) is rare malignant tumor constituting 0.5-0.9% of all soft tissue sarcomas in adults and 0.8-1.85 of those in children. Published series on presentation and treatment outcome of this sarcoma are scarce. Aim and Objectives: We present our experience in treating patients with this disease. The clinical features, diagnosis, treatment and prognostic factors of patients with ASPS were studied. Materials and Methods: Between 1975 and 2008, 23 patients with alveolar soft part sarcoma were treated in our hospital. We collected the files and described their characteristics, therapy and course. Reslts: The study group consisted of 11 male and 12 female patients, the median age at diagnosis was 24 years (13-66 years). The primary site was the lower extremity (n=13), upper extremity (n=4), head and neck (n=2), abdominal wall (n=1), chest wall (n=1), back (n=1) and flank (n=1). The median follow-up was 110(2-301) months. The median survival was 48 months with an overall 5-year survival of 62%. At time of diagnosis 5 patients already had metastases. Fifteen patients (65%) suffered from lung metastasis. Among them 9 of 15 patients had lung metastasis at the initial diagnosis, 6 patients developed lung metastasis later. Median survival after diagnosis of metastases was 38.5 (1-101) months. Conclusion: Alveolar soft part sarcoma is found especially in young adults. When diagnosed it is often metastasized with a poor prognosis. However, with adequate treatment, long-term survival is possible.