JSOA Online

Alveolar soft part sarcoma (ASPS) is a rare soft tissue sarcoma that most commonly arises in the deep soft tissues of the lower extremities of adults. Median survival of patients with metastatic ASPS has been reported to be from 3 to 3.3 years. The time between detection of metastases and death varies from 10 months to 6.2 years. In this article, a case of an 11-year-old male with primary ASPS of the right forearm is presented. Successful long-term local control of the primary tumor was achieved with wide margin surgical resection and adjuvant radiation therapy. Three years after diagnosis, the patient developed pulmonary metastases. Chemotherapy was unsuccessfully used to control the metastatic disease. Despite that, the patient survived longer than expected, and passed away 9 years after the detection of pulmonary metastases. This time to death after the development of metastases vastly exceeded the previously reported survival rates of patients with metastatic ASPS. (Journal of Surgical Orthopaedic Advances 15(4):209–213, 2006)