Pulmonary resection and chemotherapy for metastatic alveolar soft-part sarcoma

Alveolar soft-part sarcoma (ASPS) is an unusual tumor of soft tissues; it has invariably ended in death from disseminated disease, and the lung has been the most common site of metastasis. We present a patient with ASPS with bilateral pulmonary metastases who achieved a complete response after bilateral thoracotomies with removal of all gross disease and after combination chemotherapy including vincristine, actinomycin D, cyclophosphamide, and doxorubicin. The patient has now been followed for five years since the appearance of the metastases and has been off therapy for the past 34 months. He shows no evidence of disease. We suggest that multimodality therapy is a reasonable approach in patients with ASPS and pulmonary metastases and that such therapy has the potential for improving survival.