Tumors of central and peripheral nervous system associated with inherited genetic syndromes

Pediatr Neurosurg. 2012;48(5):271-85. doi: 10.1159/000351546. Epub 2013 Jun 18.

Abstract

There are several genetic syndromes that predispose to the development of tumors of the nervous system. In the present study, we provide a review of the tumors that are associated with neurofibromatosis type 1, neurofibromatosis type 2, tuberous sclerosis complex, von Hippel-Lindau disease, Li-Fraumeni syndrome, Cowden disease, Turcot syndrome, nevoid basal cell carcinoma syndrome (Gorlin syndrome) and rhabdoid predisposition syndrome, which are the most common.

Publication types

  • Review

MeSH terms

  • Animals
  • Brain Neoplasms / diagnosis
  • Brain Neoplasms / epidemiology
  • Brain Neoplasms / genetics
  • Central Nervous System Neoplasms / diagnosis*
  • Central Nervous System Neoplasms / epidemiology
  • Central Nervous System Neoplasms / genetics*
  • Colorectal Neoplasms / diagnosis
  • Colorectal Neoplasms / epidemiology
  • Colorectal Neoplasms / genetics
  • Humans
  • Li-Fraumeni Syndrome / diagnosis
  • Li-Fraumeni Syndrome / epidemiology
  • Li-Fraumeni Syndrome / genetics
  • Neoplastic Syndromes, Hereditary / diagnosis
  • Neoplastic Syndromes, Hereditary / epidemiology
  • Neoplastic Syndromes, Hereditary / genetics
  • Neurofibromatosis 1 / diagnosis
  • Neurofibromatosis 1 / epidemiology
  • Neurofibromatosis 1 / genetics
  • Peripheral Nervous System Neoplasms / diagnosis*
  • Peripheral Nervous System Neoplasms / epidemiology
  • Peripheral Nervous System Neoplasms / genetics*
  • Syndrome
  • Tuberous Sclerosis / diagnosis
  • Tuberous Sclerosis / epidemiology
  • Tuberous Sclerosis / genetics
  • von Hippel-Lindau Disease / diagnosis
  • von Hippel-Lindau Disease / epidemiology
  • von Hippel-Lindau Disease / genetics

Supplementary concepts

  • Turcot syndrome