Neurilemmomas are relatively uncommon lesions most commonly encountered in the nerve sheath originating from Schwann cells. They are rarely found in the oral cavity and are characterized by solitary occurrence, slow, persistent growth and smooth surface, along with inconsistent clinical aspects depending on the nerve origin. Microscopically, the lesion typically comprises of alternating regions of hypercellularity and hypocellularity such as Antoni A and Antoni B arrangements, respectively. Here, we present a case of a benign spindle cell tumor of the mandible reflecting a case of intraosseous neurilemmoma in a 33-year-old female patient. Immunopositivity with S-100 and vimentin clearly indicates the lesion to be a spindle cell tumor with neural involvement. The prognosis was favorable; complete surgical removal was the treatment of choice with no recurrence noted. The present case further magnifies the importance of neurilemmoma and further aids in the differential diagnosis of several other benign soft tissue tumors in the oral cavity.
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