Purpose: The aim of this study was to evaluate the clinicopathologic characteristics and the results of surgical treatment for alveolar soft part sarcoma (ASPS)
Methods: The clinicopathologic characteristics and surgical treatment results were analyzed retrospectively by reviewing the medical records of 11 patients and interviewing them. Overall survival (OS) was calculated using the Kaplan-Meier method.
Results: Of the 11 patients, 5 men and 13 women, the median age at diagnosis was 27 years and the mean tumor diameter was 6.0±1.9 cm. Six patients without distant metastasis at the time of diagnosis were treated with radical resection, and the other 5 with distant metastasis underwent palliative surgery or biopsy only, followed by chemo-radiation therapy. The onset of distant metastasis (40.0±13.5 months) was similar to the onset of local recurrence (43.3±22.4 months) after a radical operation. The most common site of metastasis was the lung, followed by the brain and bone. The median survival was 52 months and the 5-year OS was 27.2%. The 2-year OS in stage Ⅳ disease was 27.7%. The 5-year OS and the disease free survival among the patients with localized disease were 53.3% and 0%, respectively (P=0.043).
Conclusion: Complete resection is the primary therapeutic option for ASPS. Close, long-term follow-up is recommended in order to detect recurrence, particularly in the lung, bone and brain. Long term survival can be achieved with aggressive surgery for local recurrence and even distant metastasis.
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