Current Neurology and Neuroscience Reports

, Volume 12, Issue 2, pp 153–164

Syndromes Predisposing to Pediatric Central Nervous System Tumors: Lessons Learned and New Promises

Pediatric Neurology (R Packer, Section Editor)

DOI: 10.1007/s11910-011-0244-5

Cite this article as:
Villani, A., Malkin, D. & Tabori, U. Curr Neurol Neurosci Rep (2012) 12: 153. doi:10.1007/s11910-011-0244-5


Central nervous system (CNS) neoplasms are a leading cause of morbidity and mortality among children with cancer. In contrast to adults, a genetic basis for brain tumors is relatively common in children. A child harboring a germline mutation in a cancer-related gene will be predisposed to develop CNS tumors. These cancer predisposition syndromes are rare but pose overwhelming clinical and psychosocial challenges to families and the treating team. Recent significant advances in our understanding of the biological processes that govern these genetic conditions combined with international efforts to define and treat clinical aspects of these tumors are transforming the lives of these individuals. In this article, we summarize recent progress made for each of the major CNS tumor syndromes. We discuss the biological and clinical relevance of such advances, and suggest a comprehensive approach to a child affected by a predisposition to brain tumors.


Brain tumorCancer predispositionChildCentral nervous system tumors

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  1. 1.Division of Haematology/OncologyThe Hospital for Sick ChildrenTorontoCanada
  2. 2.Genetics and Genome Biology ProgramThe Hospital for Sick ChildrenTorontoCanada
  3. 3.The Arthur and Sonia Labbat Brain Tumor Research CentreThe Hospital for Sick ChildrenTorontoCanada