Multiple spinal schwannomas in absence of neurofibromatosis (Schwannomatosis) – A rare condition: Review with case report

Abstract

Schwannomas are benign, slow-growing tumors originating from sensory rootlets. Schwannomatosis is a distinct clinical syndrome characterized by the presence of multiple schwannomas in the spine with the absence of typical features suggestive of either neurofibromatosis 1 (NF1) and neurofibromatosis 2 (NF2). It is essential to name schwannomatosis as a distinct syndrome on the basis of genetic and molecular studies. Management in schwannomatosis is surgical removal if symptomatic, and if asymptomatic it is better to follow-up with regular screening. The author reports here a rare case of a 33-year-old male patient, who had a history of being operated for dorsal spine schwannoma at D6 level 7 years back. Now, after 7 years, the patient presented with back pain, moderate to severe, associated with difficulty in walking. After proper history, clinical and radiological evaluation the patient was found to be having multiple lesions at D12 and L3 level with no family history, and on histopathology, both lesions were found to be schwannomas. Hence, this case was diagnosed as a case of multiple schwannomas without any features suggestive of either NF1 or NF2 (schwannomatosis). Regular follow-up is very essential in every case suggestive of schwannoma, as new multiple lesions can develop at any time, after years as discussed in our case.