Lingual alveolar soft part sarcoma with absent TFE3 rearrangement.

BACKGROUND: Lingual ASPS is extremely rare and aggressive tumor. rearrangement is typically detected in ASPS patients using FISH analysis. AIM: To present the clinical, histopathological, and radiological features of lingual Alveolar Soft Part Sarcoma. METHOD: A 30-year-old male presented with a painless, slowly growing mass of the tongue. Initial impression was of benign vascular lesion. Later, the patient became symptomatic as the mass progressed in size, which necessitated further investigations. RESULT: A lip-split, mandibulotomy was performed for the excision of the tumor and revealed an alveolar soft part sarcoma with PAS-positive, diastase resistant intracytoplasmic granules. However, molecular analysis using FISH was negative for TFE3 rearrangement. Patient underwent partial glossectomy with postoperative radiotherapy. CONCLUSION: Clinical and pathological correlation of ASPS is very useful to reach a proper diagnosis.