Alveolar soft part sarcoma is a rare malignant soft tissue neoplasm of uncertain histogenesis and aggressive clinical behavior. Alveolar soft part sarcoma arises in the head and neck in 27% of cases, with 25% of head and neck cases occurring in the tongue. Herein the case of a pediatric patient diagnosed with alveolar soft tissue sarcoma of the tongue, who received surgical treatment with total resection of the lesion and chemotherapy without radiotherapy, is presented.
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