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Recent advances in embryonal tumours of the central nervous system

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Abstract

Introduction

Embryonal tumours of the central nervous system (CNS) are the commonest malignant paediatric brain tumours. This group includes medulloblastomas, supratentorial primitive neuroectodermal tumours, atypical teratoid/rhabdoid tumours, ependymoblastomas, and medulloepitheliomas. Earlier, all these tumours were grouped under a broad category of primitive neuroectodermal tumours (PNETs). However, the current WHO classification (2000) separates them into individual types based on significant progress in the understanding of their distinctive clinical, pathological, molecular genetic, histogenetic, and behavioural characteristics. Furthermore, advances in histopathology and molecular genetics have shown great promise for refining risk assessment in these tumours, especially medulloblastomas, thus providing a more accurate basis for tailoring therapies to individual patients. Correlation of histological changes with genetic events has also led to a new model of medulloblastoma tumorigenesis.

Review

This review presents an updated comparative profile of these tumours, highlighting the clinical and biological relevance of the recent advances.

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Acknowledgements

The authors wish to thank the Indian Council of Medical Research for funding a research project on medulloblastomas. The secretarial assistance of Mr. Kamal is sincerely acknowledged.

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  1. Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, 110029, India

    Chitra Sarkar, Prabal Deb & Mehar Chand Sharma

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  1. Chitra Sarkar
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  2. Prabal Deb
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Correspondence to Chitra Sarkar.

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Sarkar, C., Deb, P. & Sharma, M.C. Recent advances in embryonal tumours of the central nervous system. Childs Nerv Syst 21, 272–293 (2005). https://doi.org/10.1007/s00381-004-1066-4

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