ABSTRACT

In the vast majority of cases no aetiological factors can be identified. Several associations have been identified but these conditions are them-

selves very rare. Genetic predisposition to CNS tumours has been identified in the following rare syndromes:

■ neurofibromatosis – neurofibroma, and neurofibrosarcoma, optic nerve glioma, ependymoma, meningioma

■ tuberose sclerosis – glioma and hamartoma ■ Von Hippel-Lindau syndrome – cerebellar

haemangioblastoma ■ Li-Fraumeni syndrome – gliomas ■ Gorlin’s syndrome – medulloblastoma.