Alveolar soft part sarcoma (ASPS) is a rare, malignant tumor of uncertain histogenesis that has no benign counterpart. In some cases, a structural rearrangement of chromosome 17 involving band q25 has been reported. The neoplasm occurs most frequently in female adolescents and young adults, where it arises predominantly in the extremities. In contrast, the most common sites of occurrence in infants and children are the orbit (41%) and the tongue (25%). The primary therapeutic option is a complete surgical excision. Because of the indolent growth and lack of pain associated with the mass, 20% of patients have metastases at the time of initial diagnosis. Median survival time reported for all sites of the body is 79 months. When ASPS presents in the tongue region, however, the patients involved are usually children and have a better prognosis than patients affected in the extremities. The utility of adjuvant chemotherapy or radiation therapy in children is open to question. Because metastases may occur after several decades, children with ASPS should be followed throughout adolescence and well into adulthood. Only 10 cases of ASPS occurring in the tongues of children younger than 5 years of age were indexed by MEDLINE between 1952 and 2006. Here, we describe the 1st case consistent with typical ASPS of the tongue in 15 years at our hospital. The patient is a 2-year-old girl who has been disease-free for 32 months.

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