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Granular cell tumor (GCT) is an uncommon mostly benign neoplasm named after Abrikossoff. This mesenchymal neoplasm supposedly arises from the Schwann cells. Most commonly presents as asymptomatic swelling in the gastrointestinal tract and skin. Histologically in the dermis polyhedral cells with round vesicular nucleus with eosinophilic cytoplasm is seen. Here, we present a case of a malignant GCT in a 45-year-old male over the upper back which on biopsy revealed neuron-specific enolase-positive tumor cells and the patient was treated with S-plasty with wide excision, there was no recurrence till 1 year.

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