Abstract:
Objective This work aims to analyze the clinical features and curative effects of 42 cases with alveolar soft-part sarcoma (ASPS).
Methods Data of 42 patients with ASPS, who received treatment at this hospital, during the period May 1962 to Au- gust 2008, were reviewed. The age of the patients ranged from 9 years to 56 years (median 27). There were 23 females and 19 males in the cases. The major site of the primary cancer was the limbs (83.3 %, n = 35). The median size of the primary tumor was 4.2 cm. Five patients (12 %) presented metastatis. Wide local excision of the primary cancer was performed in 38 of the 42 patients.
Results After a median follow-up of 57 months (range: 18 months to 187 months), the overall survival rate at 5, 10, and 15 years were 81.7 %, 63.6 %, and 31.8 %, respectively. Recurrence or metastatis occurred in 27 patients (64.3 %) within the follow-up period. Local recur- rence occurred in only 1 of the cases, local recurrence combined with metastasis in 3, and metastatic disease alone in 23. Pulmonary me- tastasis was found in 21 patients, brain metastasis in 7, and osseous metastasis in 5. The median survival time after diagnosis of distant metastasis was 36 months (ranging from 17 months to 82 months). Chemotherapy using a variety of regimens was performed in 20 of the patients who suffered distant metastasis. Six patients also underwent metastasectomy for pulmonary metastasis. Response to chemo- therapy was recorded in 14 patients, among which stable disease was seen in 6 and progressive disease in 8.
Conclusion ASPS dis- plays a relatively indolent clinical course and features a younger age range. Surgery is the mainstay of therapy. The ultimate prognosis of ASPS is dissatisfactory and is often characterized by late metastasis to the lung, bone, or brain.
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