Child's Nervous System

, Volume 21, Issue 4, pp 272–293

Recent advances in embryonal tumours of the central nervous system

Review Paper

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.

Keywords

Central nervous system Embryonal tumours Histopathology Molecular genetics Prognostic factors Therapeutic modalities 

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Copyright information

© Springer-Verlag 2005

Authors and Affiliations

  • Chitra Sarkar
    • 1
  • Prabal Deb
    • 1
  • Mehar Chand Sharma
    • 1
  1. 1.Department of PathologyAll India Institute of Medical SciencesNew DelhiIndia

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