A CASE OF INTRA-EXTRADURAL CERVICAL SCHWANNOMA IN A CHILD WITH NEUROFIBROMATOSIS - TYPE 1

Dr. A. Susmitha, Dr. V. Anurekha, Dr. S. Amudhadevi, *Dr. K. S. Kumaravel, P. Sampathkumar, Dr. L. Sankar

ABSTRACT

Neurofibromatosis type 1 (NF1) is a rare autosomal dominant inherited disorder caused by mutation in NF-1 gene which is located in chromosome 17q11.2. Loss of the neurofibromin, the inactivator of protooncogen RAS, leads to an increase in incidence of tumors. Thus, patients are predisposed to have any central nervous system tumors, especially gliomas, which are the most common neoplasms in NF1. Spinal tumors are relatively rare. Meningiomas and Schwannomas are the two most common intradural extramedullary tumors associated with neurofibromatosis. Schwannoma is benign slow growing nerve sheath tumour. Schwanomas are mostly intradural extramedullary, and upper cervical in location. About 75% of schwannomas are intradural, 10% intra-extradural, and the rest 15% are extradural. We here report a 10-year-old boy with NF-1 presented with weakness of all four limbs and neck pain and was found to have MRI demonstrated intra-extradural mass, suggestive of schwannoma. Intraoperative findings found to be multi lobulated intradural extramedullary tumor extending with extradural component. The diagnosis of schwannoma was confirmed by Histo Pathological Examination which demonstrated Verocay bodies and with Immuno Histo Chemistry studies which demonstrated the presence of strong S-100 proteins. These are benign tumors and complete functional recovery can be obtained after complete excision.

Keywords: Meningiomas and Schwannomas.