Hereditary Syndromes Associated with Brain Tumors. Literature Review.

Tumors of the central nervous system (CNS) represent 2% of all neoplasms. Low-prevalence hereditary syndromes constitute only a small proportion of all cases of primary tumors of the central nervous system. Most of these syndromes have autosomal-dominant inheritance, except in the case of Turcot syndrome type 1 which has an autosomal recessive inheritance pattern. It is important in daily neurosurgical practice to be able to recognize these hereditary syndromes in order to apply the best therapeutic alternative in these patients.