Familial Central Nervous

Abstract

THE FAMILIAL occurrence of central nervous system (CNS) neoplasia is a rare event. Hauge and Harvald 1,2 found no increased risk to relatives of propositi with glioma, meningioma, or medulloblastoma. Van der Wiel, 3 however, found in his series of 100 patients with central glioma that 1 of 200 parents and 2 of 500 sibs were similarly affected; a risk he felt to be four times greater than expected. Several reviews 4,5 have compiled isolated instances of cerebral tumors in twins and in blood relatives totaling about 50 reports in all. From these reports a genetic predisposition is suggested. The occurrence of a variety of CNS neoplasms associated with von Recklinghausen's disease (neurofibromatosis) has been documented in the literature. 6 The purpose of this report is to describe a truly remarkable family in which eight members have been affected by CNS tumors. Only minor evidence of peripheral von Recklinghausen's