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Tumors of the Central Nervous System, Volume 9 pp 77–83Cite as

Supratentorial Primitive Neuroectodermal Tumor: Biology

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Part of the book series: Tumors of the Central Nervous System ((TCNS,volume 9))

Abstract

Supratentorial primitive neuroectodermal tumours (StPNETs) share similar histopathological features to medulloblastomas, and have accordingly been treated alongside their infratentorial counterparts but have a significantly inferior prognosis. The biological study of these tumours has been limited, but a number of crucial insights have recently been identified. The cytogenetic features of pineal and non-pineal StPNETs differ, and chromosome 17p loss, which characterises up to 40% of medulloblastomas is not a feature of StPNET disease. Disruption of developmental pathways including Wnt signalling, Notch and Sonic hedgehog pathways occur in a small subset of StPNET tumours. p53 pathway disruption in contrast, and in distinction from medulloblastoma, is a frequent feature of StPNET. Genetic features including mutations inIDH1and carrying a discrete amplification on chromosome 19 (19q13.41) are associated with StPNET clinical characteristics. Further studies are required to facilitate the exploitation of these genetic features to update and enhance the current classification, treatment and outcome in this disease.

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Authors and Affiliations

  1. Pediatric Oncology, Great North Children’s Hospital, Royal Victoria Infirmary, Queen Victoria Road, Newcastle upon Tyne, NE1 4LP, UK

    James Hayden

  2. Department of Oncology, Alder Hey Children’s Hospital, Eaton Road, Liverpool, L122AP, UK

    Barry Pizer

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  1. James Hayden
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  2. Barry Pizer
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Correspondence to Barry Pizer .

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Editors and Affiliations

  1. , Kean University, Room 213, Library building, Morris Avenue 1000, Union, 07083, New Jersey, USA

    M.A. Hayat

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Hayden, J., Pizer, B. (2012). Supratentorial Primitive Neuroectodermal Tumor: Biology. In: Hayat, M. (eds) Tumors of the Central Nervous System, Volume 9. Tumors of the Central Nervous System, vol 9. Springer, Dordrecht. https://doi.org/10.1007/978-94-007-5488-1_10

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  • DOI: https://doi.org/10.1007/978-94-007-5488-1_10

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