[Cerebral tumors in children with neurofibromatosis type 1].

Abstract

Twenty-eight children (mean age 8 years) with neurofibromatosis type 1 (NF1) and cerebral tumor were studied from 1975 to 1992 (mean follow-up 8.1 years) considering the biological behaviour of the tumor and the patient's quality of life, in order to identify retrospectively the best management. All, except one, tumors were benign gliomas, 76% of the optic nerve/chiasm (NCO), just 10% infratentorial. Sixteen children (57%) did not receive any treatment, 2 radiotherapy (RT) only and 4 symptomatic treatment only; in 6 patients the tumor resection was performed. 92% of the 25 survivors had sufficient autonomy in daily life at last follow-up. Considering the risk of cerebral tumors in patients with NF1, we conclude that cerebral magnetic resonance should be performed also in the asymptomatic ones. If neuroradiological findings are characteristic of benign glioma, histologic confirmation seems unnecessary. Surgical resection is recommended only in tumors confined to a single optic nerve, with severe or progressive symptoms. In chiasmatic tumors we suggest partial resection or symptomatic treatment only with close clinical and radiological observation. RT is only recommended if there is unequivocal evidence of tumor progression. Chemotherapy can delay the use of RT in very young children. Cerebral tumors different from NCO gliomas seem to have a similar natural history in patients with or without NF1 and therefore the management should be the same for both groups.