Surgical Treatment for the Alveolar Soft Part Sarcoma

Ahn, Soo Min; Oh, Jung Tak; Choi, Seung Hoon

Abstract

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 6 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.