[Basic science and clinical aspects of familial brain tumors]

Familial brain tumor syndromes include neurofibromatosis 1, neurofibromatosis 2, tuberous sclerosis, von Hippel-Lindau disease, and multiple endocrine neoplasia type 1, which are hereditary diseases. In these diseases, various tumors develop because of alterations in tumor suppressor genes. Familial brain tumors are mostly benign, but some are malignant. Familial brain tumor syndromes are diagnosed on the basis of guidelines for clinical diagnosis and by DNA test. Unlike sporadic brain tumors, familial brain tumors occur at multiple sites; therefore, a single operation is often not sufficient for treating familial brain tumors, and it is often necessary to treat lesions in other organs. Surgical indications should be considered more prudently for familial brain tumors than for sporadic brain tumors.