Child's Nervous System

, Volume 30, Issue 1, pp 47–64 | Cite as

Familial syndromes associated with intracranial tumours: a review

  • Adrianna M. Ranger
  • Yatri K. Patel
  • Navjot Chaudhary
  • Ram V. Anantha
Review Paper

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 

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Copyright information

© Springer-Verlag Berlin Heidelberg 2013

Authors and Affiliations

  • Adrianna M. Ranger
    • 1
  • Yatri K. Patel
    • 1
  • Navjot Chaudhary
    • 2
  • Ram V. Anantha
    • 3
  1. 1.Department of Clinical Neurological SciencesWestern UniversityLondonCanada
  2. 2.Department of NeurosurgeryStanford School of MedicineStanfordUSA
  3. 3.Department of Surgery, Schulich School of Medicine and DentistryWestern UniversityLondonCanada

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