Alveolar soft-part sarcoma (ASPS) is a rare and often fatal tumor. Overall survival rates have been reported to be 62% after 5 years, 42% after 10 years, and 18% after 20 years. ASPS accounts for 5% of all pediatric soft-tissue sarcomas other than rhabdomyosarcomas. In children, ASPS rarely occurs in the oral cavity, and to the best of our knowledge, only 12 cases of ASPS of the tongue occurring during the first decade of life have been previously reported in the literature. Because of the rarity of lingual ASPS in children, no specific treatment protocols have been developed, which makes its management difficult. We report a new case of lingual ASPS in a young child. Our patient was a 17-month-old girl whose tumor was located at the base of the tongue. She was successfully treated with surgical excision without adjuvant radiation or chemotherapy. Postoperatively, she has remained disease-free during 4 years of follow-up.
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