Alveolar soft-part sarcoma: Case demonstrating principles for uncommon vascular lesions

Alveolar soft-part sarcoma (ASPS) is an uncommon malignancy that may present in a manner similar to benign vascular tumors.

A 6-year-old boy with autism spectrum disorder was referred to the Johns Hopkins All Children's Hospital vascular anomalies clinic for the evaluation of a tongue mass.

Prior to the presentation, at 5 years of age, neck computed tomography (CT) was performed. This showed a well circumscribed, enhancing mass at the anterior aspect of the tongue. The radiologic impression was that this lesion was most likely a hemangioma. Two years later, the patient was evaluated in the vascular anomalies clinic. At that examination a 2-3 cm swelling was noted on the dorsal aspect of the tongue. The mass was fleshy and firm with discrete borders. Handheld Doppler examination indicated a high-flow lesion. The patient underwent an excisional biopsy. The lesion was identified as an alveolar soft-part sarcoma based on pathologic characteristics.

Familiarity with common vascular tumors and malformations allows providers to diagnose the majority of these lesions on a combination of clinical history and physical examination. Atypical and combined lesions do benefit from imaging to help characterize and aid in the differential diagnosis. Biopsy enables definitive diagnosis but is necessary in the minority of cases. When in doubt, referral to a specialized, multidisciplinary vascular anomalies clinic will ensure that these patients receive management for this challenging collection of conditions.