Review Paper

Child's Nervous System

, Volume 30, Issue 1, pp 47-64

First online:

Familial syndromes associated with intracranial tumours: a review

  • Adrianna M. RangerAffiliated withDepartment of Clinical Neurological Sciences, Western University Email author 
  • , Yatri K. PatelAffiliated withDepartment of Clinical Neurological Sciences, Western University
  • , Navjot ChaudharyAffiliated withDepartment of Neurosurgery, Stanford School of Medicine
  • , Ram V. AnanthaAffiliated withDepartment of Surgery, Schulich School of Medicine and Dentistry, Western University

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Abstract

Background

Most cancers of the central nervous system (CNS) occur sporadically in the absence of any known underlying familial disorder or multi-systemic syndrome. Several syndromes are associated with CNS malignancies, however, and their recognition has significant implications for patient management and prognosis. Patients with syndrome-associated CNS malignancies often have multiple tumours (either confined to one region or distributed throughout the body), with similar or different histology.

Objective

This review examines syndromes that are strongly associated with CNS cancers: the phakomatosis syndromes, familial syndromes such as Li–Fraumeni and familial polyposis syndromes and dyschondroplasia.

Keywords

Central nervous system malignancies Familial syndromes Phakomatosis syndromes Dyschondroplasia