Surgical Treatment of Alveolar Soft Part Sarcoma of the Extremity: Results of at Least 5 Years of Follow-up

Abstract

Objective:Alveolar soft part sarcoma (ASPS) is a rare soft tissue tumor that usually affects young patients. Because of the rarity of the disease, most reports relating to ASPS are in the form of case reports or small series.Methods:We performed a retrospective study to evaluate the clinic features, treatment and outcome in a consecutive series of patients with localized or metastatic ASPS between 2004 and 2014. Demographics, tumor sizes, sites of tumor and extent of disease, treatments provided, progression, and overall survival were evaluated.Results:Total of 8 patients were identified. The clinical assumptive diagnosis of the doctor making first medical examination was benign soft tissue tumor like hemangioma in 3 cases (37.5%), delaying treatment. The most common loca¬tion of primary tumor was the thigh. The median diameter of the mass was 136.8±76.0 mm (range=4-572 mm). Median overall follow-up was 64 months.Conclusion:It was found that age greater than 28 years at the time of diagnosis, and the non-thigh placement of the tumor on the limb were found to increase the risk of lung metastasis. It was found that 37.5% of the patients underwent inadequate operation with the pre-diagnosis of vascular-related benign tumors. So, a comprehensive preoperative evaluation for the differential diagnosis of ASPS is needed when interfering with hemangioma-like vascular associated tumors.

Keywords

• alveolar soft part sarcoma
• extremity
• thigh
• prognostic factors
• haemangioma