NEUROLOGIC AND OTHER DISORDERS IN RELATIVES OF PEDIATRIC-PATIENTS WITH CNS TUMORS

Abnormalities of embryogenesis and nervous system development may caus e or contribute to the development of childhood brain tumors, To ident ify genetic or environmental factors that may be associated with etiol ogies of childhood central nervous system tumors, we examined family h istories of 165 children with such tumors for the presence of neurolog ic disorders, including neural tube defects, mental retardation, seizu res, and central nervous system tumors, as well as other cancers and b irth defects, Only 1 patient, with the neurofibromatosis-Noonan syndro me, was confirmed to have an underlying syndromic diagnosis associated with central nervous system tumorigenesis. Families of 2 probands wit h posterior fossa primitive neuroectodermal tumors reported relatives with olivopontocerebellar atrophy, Although increased incidences of st udy disorders were not identified in this population, it is possible t hat within individual families one or more of these disorders is relat ed to childhood central nervous system tumorigenesis.