Recent advances in embryonal tumours of the central nervous system.
BACKGROUND
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.


METHODS
This review presents an updated comparative profile of these tumours, highlighting the clinical and biological relevance of the recent advances.
Sarkar
Chitra
C
Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi. drchitrasarkar@yahoo.com
Deb
Prabal
P
Sharma
Mehar Chand
MC
D017677:Age Distribution; D000818:Animals; D016543:Central Nervous System Neoplasms / Q000196:embryology* / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D008527:Medulloblastoma; D009373:Neoplasms, Germ Cell and Embryonal / Q000145:classification / Q000235:genetics* / Q000473:pathology*; D012737:Sex Factors; D014157:Transcription Factors / Q000235:genetics
D014157:Transcription Factors