Abstract
A 64-year-old black man had a primary alveolar soft part sarcoma of the tongue. It is unusual for this rare tumor to develop in this site in the elderly age group. The patient survived nearly 3 years with disease. When the patient expired, a complete autopsy was performed. The biopsy material of the primary tumor showed characteristic light microscopic appearance, intracellular periodic acid-Schiff positive inclusions after diastase reaction, and electron microscopic presence of crystals. Immunohistochemistry for neuron-specific enolase was positive. The autopsy confirmed widespread visceral and lymphatic metastases. The atypical features of this case, including the site of the primary lesion, age of the patient at diagnosis, immunohistochemistry of the tumor, and natural history of this neoplasm, suggest that alveolar soft part sarcoma may behave differently when arising in an extraskeletal site, and could possibly have a different histogenesis from the typical alveolar soft part sarcoma found in a younger population.
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