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.