Genetics, prognosis and therapy of central nervous system tumors.

Tumors of the central nervous system (CNS) are common causes of morbidity and mortality. These tumors can occur sporadically or in individuals with genetic disorders predisposing to cancer development. Such syndromes include neurofibromatosis type 2, neurofibromatosis type 1, Li-Fraumeni syndrome, as well as von Hippel-Lindau disease, tuberous sclerosis, and Turcot syndrome. There may also be familial syndromes resulting in glioma or meningioma alone, but these are not well understood. Development of sporadic gliomas is accompanied by a number of molecular genetic alterations, including activation of dominant oncogenes and inactivation of tumor suppressor genes. Some of these alterations may be associated with progression of gliomas to their most malignant form, glioblastoma multiforme. However, at this time molecular genetic analysis of gliomas does not provide better prognosis than histopathological staging. Recently, experimental treatments of gliomas in rodents, using gene therapy, have been reported. Results of these studies have been promising, and these techniques may represent a future direction for therapy in humans.