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Lingual alveolar soft part sarcoma with absent TFE3 rearrangement
Saeed Alshahrani1, Abdullatif Khan2
1College of Medicine, King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia.
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.
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