Review Paper

Child's Nervous System

, Volume 21, Issue 4, pp 272-293

Recent advances in embryonal tumours of the central nervous system

  • Chitra SarkarAffiliated withDepartment of Pathology, All India Institute of Medical Sciences Email author 
  • , Prabal DebAffiliated withDepartment of Pathology, All India Institute of Medical Sciences
  • , Mehar Chand SharmaAffiliated withDepartment of Pathology, All India Institute of Medical Sciences

Rent the article at a discount

Rent now

* Final gross prices may vary according to local VAT.

Get Access

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