Alveolar soft part sarcoma: clinical presentation, treatment and outcome in a series of 19 patients

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.

We performed a retrospective study to evaluate the clinicopathologic features, treatment, outcome and pattern of treatment failure in a consecutive series of patients with localized or metastatic ASPS between 1996 and 2011. Demographics, tumor sizes, sites and extent of disease, treatments provided, progression-free survival, and overall survival were evaluated.

A total of 19 patients were identified. The clinical assumptive diagnosis of the first medical examination doctor was benign soft tissue tumor in 5 cases (26%) and benign hemangioma in 4 cases (21%), delaying treatment. The most common location of primary tumor was the thigh. The median diameter of the mass was 55 mm (range, 10 to 130 mm). An R0 resection was obtained in 11 cases. Adjuvant radiotherapy was delivered in 8 cases; postoperative systemic chemotherapy was delivered in 10 cases. Eight out of 15 patients (53%) exhibited metastases either at presentation or later. Median overall follow-up was 54 months.

The treatment principle for alveolar soft tissue sarcoma is massive resection, and when the surgical margin is questionable, radiation treatment can be added for prevention of local recurrence. Also, due to discovery of metastases and local recurrence, even after 5 years of general treatment, outpatient department follow-up is needed, and we need to keep in mind that lung, intracranial, and bone metastases are common.