Alveolar soft part sarcoma of the tongue

Alveolar soft part sarcoma is a rare malignancy. To our knowledge, 13 cases in the head and neck have been reported previously. It occurs more commonly in the extremities, where it is associated with a poor prognosis. Its clinical course resembles that of adenoid cystic adenocarcinoma in that late metastases often cause death in 10 to 15 years. A 5-year-old girl with alveolar soft part sarcoma was successfully treated with partial glossectomy and is free of disease at two-year follow-up.