Abstract
Schwannomatosis is a recently recognized distinct form of neurofibromatosis (NF). It is a rare condition, the incidence of which varies between 1/400,000 and 1/1.7 million. An important feature of schwannomatosis is the presence of multiple intracranial, spinal, and peripheral schwannomas in the absence of acoustic neuromas. Schwannomatosis presenting with intraosseous schwannoma of the mandible is even rarer, and only a few cases have been reported. It usually affects individuals in the third to fifth decade of life. Usually, it is sporadic in origin, but in 20% of patients, it can be familial. As a diagnostic criterion, NF2 gene is not involved in schwannomatosis. We report a case of a 48-year-old male presenting with facial pain and difficulty in chewing, and subsequent development of spastic paraplegia. Magnetic resonance imaging scan of head and neck revealed mass lesion involving infratemporal region on the left side, intraosseous lesion of the mandible, and multiple mass lesions in the neck. Acoustic nerves were not involved. Mutagen-induced chromosome sensitivity analysis test suggested no predisposition for malignancy. His clinical features are suggestive of schwannomatosis, which is a recently recognized distinct form of NF.