The neurosurgical aspects of neurofibromatosis 2: diagnosis and management.

NF-2 is an extremely rare form of neurofibromatosis (NF) characterized by central system (CNS) neural crest-derived tumors and frequently cafe au lait spots (CLS). The purpose of this study was to report the clinical and imaging findings of seven patients with this disorder and to stress that value of surgical treatment in its management. Seven patients between 8 and 32 years of age who had NF-2 were included in the study. Clinical charts, surgical and pathological findings, and imaging studies were reviewed retrospectively. Patients were followed up for to 142 months. Clinical evaluation and neuroimaging studies detected the clinical criteria of NF-2 in all patients. Two deaths occurred after surgical intervention in our series. None of the patients suffered from recurrent tumor following surgery. Our results show that NF-2 is an uncommon entity which has a good prognosis after surgical intervention, in spite of the presence of multiple cranial and/or spinal lesions.