2001 CTOS Annual Meeting Posters— Surgery

ALVEOLAR SOFT PART SARCOMA: A REPORT OF 15 CASES.
Serge van Ruth,  Frits van Coevorden,  Bin B.R. Kroon
Department of Surgical Oncology Netherlands Cancer Institute, Amsterdam NL

METHODS: Between 1971 and 2000 15 patients with alveolar soft part sarcoma were headed in our hospital. We collected the files and described their characteristics, therapy and course.

RESULTS: The study group consisted of 8 male patients with a mean age at diagnosis of 29 years and 7 female patients with a mean age of 24 years. The primary site was the lower extremity (n=8), buttock (n=2), abdominal wall (n=1), axilla (n=1) and temple (n=1). The median follow-up was 170 (2-370) months. The median survival was 48 months with an overall 5-year survival of 38%. At time of diagnosis 5 patients already had metastases, all pulmonary localized. 5 patients developed early metastases at follow-up, after a tumor free interval of respectively 1, 1, 2, 8 and 12 months. Median survival after diagnosis of metastases was 29 (1-111) months. One is alive with evidence of disease, the other 8 died of their disease. One patient with metastatic disease died after an unexpected long survival (111 months) under various treatment schedules. Another patient with a local recurrence underwent surgery and adjuvant radiotherapy; she is still alive with no evidence of disease, 295 months after the initial diagnosis. Four other patients are alive without disease after 12, 12, 234 and 242 months. In this study chemotherapy influenced the course of the disease only incidentally.

CONCLUSION: Alveolar soft part sarcoma is found especially in young adults. When diagnosed it is often metastasized with a poor prognosis. However, with adequate local treatment, long-term survival is possible.