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

  • Pediatric Neurology (R Packer, Section Editor)
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Abstract

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.

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Disclosure

Conflicts of interest: A. Villani: none; D. Malkin: has received grant support from the Canadian Institute of Health Research; U. Tabori: has received grant support from the Canadian Institute of Health Research and BrainChild Canada.

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Authors and Affiliations

  1. Division of Haematology/Oncology, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada

    Anita Villani, David Malkin & Uri Tabori

  2. Genetics and Genome Biology Program, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada

    David Malkin

  3. The Arthur and Sonia Labbat Brain Tumor Research Centre, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada

    Uri Tabori

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  1. Anita Villani
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Correspondence to Uri Tabori.

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Villani, A., Malkin, D. & Tabori, U. Syndromes Predisposing to Pediatric Central Nervous System Tumors: Lessons Learned and New Promises. Curr Neurol Neurosci Rep 12, 153–164 (2012). https://doi.org/10.1007/s11910-011-0244-5

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