Objective: To investigate the clinical feature, suitable treatment and prognosis of alveolar soft part sarcoma.
Methods: The clinical data of 58 such patients treated at our hospital from 1967 to 2006 were retrospectively analyzed. Fifty-three patients except 6 with distant metastasis underwent surgical resection including 33 extensive resection and 19 local resection. Furthermore, 19 patients received postoperative adjuvant radiotherapy or chemotherapy.
Results: Eleven of 50 patients who underwent complete resection developed local recurrence. Thirty-One (53.4%) was found to have lung metastasis. The overall 3-, 5-, 10-year survival rate was 89.5%, 74.1% and 57.7%, respectively. The median survival time of the patients was 125 months. The 3-, 5-, 10-year survival rate was 100.0%, 81.6% and 65.3% for female patients, but it was 79.6%, 67.2% and 49.7% for male patients, respectively.
Conclusion: Alveolar soft part sarcoma usually grows slowly. Though local recurrence is rarely seen, distant metastasis is frequently observed. Lung is the most common organ of metastasis. However, survival can still be long even with lung metastasis. To achieve microscopically complete resection is quite critical to localized alveolar soft part sarcoma. No survival advantage was observed when adding adjuvant radiotherapy and/or chemotherapy. The phenomena of female patient or patient with X chromosome translocation showing better prognosis than the male or other patients needs further investigation.