Child's Nervous System

, Volume 21, Issue 4, pp 272–293

Recent advances in embryonal tumours of the central nervous system

Authors

    • Department of PathologyAll India Institute of Medical Sciences
  • Prabal Deb
    • Department of PathologyAll India Institute of Medical Sciences
  • Mehar Chand Sharma
    • Department of PathologyAll India Institute of Medical Sciences
Review Paper

DOI: 10.1007/s00381-004-1066-4

Cite this article as:
Sarkar, C., Deb, P. & Sharma, M.C. Childs Nerv Syst (2005) 21: 272. doi:10.1007/s00381-004-1066-4

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 systemEmbryonal tumoursHistopathologyMolecular geneticsPrognostic factorsTherapeutic modalities

Copyright information

© Springer-Verlag 2005