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Neuro-Oncology 1999 1(4):275-281; doi:10.1093/neuonc/1.4.275
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© 1999 by the Society forNeuro-Oncology

Brain tumors in children with neurofibromatosis: Additionalneuropsychological morbidity?

Anne E. De Winter, Bartlett D. Moore, III2, John M. Slopis, Joann L. Ater and Donna R. Copeland

The University of Texas, M.D. Anderson Cancer Center,Houston, TX 77030 (A.E.D., B.D.M., J.M.S., J.L.A., D.R.C.); and The University of Texas-Houston, Medical School, Houston,TX 77030 (J.M.S.)

2 Address correspondence and reprint requests to Bartlett D. Moore, III, Ph.D.,Division of Pediatrics, Box 87, U.T. M.D. Anderson Cancer Center, 1515Holcombe Blvd., Houston, TX 77030.


  Abstract

Neurofibromatosis type 1 is a common autosomal dominant genetic disorderassociated with numerous physical anomalies and an increased incidence ofneuropsychological impairment. Tumors of the CNS occur in approximately 15% ofchildren with neurofibromatosis, presenting additional risk for cognitiveimpairment. This study examines the impact of an additional diagnosis of braintumor on the cognitive profile of children with neurofibromatosis. Acomprehensive battery of neuropsychological tests was administered to 149children with neurofibromatosis. Thirty-six of these children had acodiagnosis of brain tumor. A subset of 36 children with neurofibromatosisalone was matched with the group of children diagnosed with neurofibromatosisand brain tumor. Although mean scores of the neurofibromatosis plus braintumor group were, in general, lower than those of the neurofibromatosis alonegroup, these differences were not statistically significant. Children in theneurofibromatosis plus brain tumor group who received cranial irradiation(n = 9) demonstrated weaker academic abilities than did children withbrain tumor who had not received that treatment. These results suggest thatneurofibromatosis is associated with impairments in cognitive functioning, butthe severity of the problems is not significantly exacerbated by thecodiagnosis of a brain tumor unless treatment includes cranialirradiation.

Received January 15, 1999; Accepted May 17, 1999


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