Outcomes of alveolar soft part sarcoma from a regional tumour unit

Introduction: We present six cases of alveolar soft part sarcoma (ASPS). All patients were identified from records at our regional institute and treated between 2000 and 2010. ASPS are slow growing highly malignant tumours, often with metastatic spread at initial presentation. Patients & Methods: The mean age of the patients was 28.5 years (21 to 36), with four males and two females. Diagnosis was based on radiological and histological features. All patients had primary surgical resection (five wide local excisions, one marginal excision for extensive widespread disease) and adjuvant radiotherapy. Results: Four lesions involved the lower limb, one involved the upper limb and one originated from the retroperitoneal space. One patient presented late with pulmonary metastases and had marginal excision of the ASPS and died 2.6 years later. Recurrence occurred in two-fifths of the remaining patients at a mean of 6 months. Conclusion: Alveolar soft part sarcoma has a poor prognosis. Our data suggests that patients are managed with an agreed disease staging protocol, radical resection and kept under close surveillance with interval imaging in a specialist bone tumour unit