SUBMANDIBULAR SCHWANNOMA IN NEUROFIBROMATOSIS-1: A RARE CASE REPORT

Schwannoma is a well encapsulated benign nerve sheath tumor of neuroectodermal origin with an indolent progression unless associated with malignancy.[1] Neurofibromatosis type 1 (NF1) is an autosomal dominant neurocutaneous syndrome that causes multiple central and peripheral nerve sheath tumors.[2] Schwannomas arise from the Schwann cells as do neurofibromas and are usually solitary except in Von Recklinghausen's disease. [3] The occurrence of a schwannoma in the head and neck region is about 25%, however submandibular gland schwannoma is a rare form of an extracranial neurogenic tumor arising from peripheral, central or autonomic nerves. [4] We report a rare case of a submandibular schwannoma in a patient with neurofibromatosis-1 which presented as a painless swelling in the submandibular region, treated by total excision of the submandibular gland. There was no postoperative deficit or recurrence within 3 months of follow up.