Show Summary Details
Page of

Embryonal and pineal tumours 

Embryonal and pineal tumours
Chapter:
Embryonal and pineal tumours
Author(s):

Roger E. Taylor

, Barry L. Pizer

, Nancy Tarbell

, Alba A. Brandes

, and Stephen Lowis

DOI:
10.1093/med/9780199651870.003.0009
Page of

PRINTED FROM OXFORD MEDICINE ONLINE (www.oxfordmedicine.com). © Oxford University Press, 2020. All Rights Reserved. Under the terms of the licence agreement, an individual user may print out a PDF of a single chapter of a title in Oxford Medicine Online for personal use (for details see Privacy Policy and Legal Notice).

date: 02 June 2020

Embryonal tumours account for 20% of paediatric central nervous system (CNS) tumours. Medulloblastoma (MB), the most frequent, arises in the cerebellum. Clinical strategies have been based on series of multi-institutional trials since the 1970s. Recent understanding of the influence of molecular/biological factors has led to subdivision into four distinct subtypes with differing clinical and prognostic profiles, on which stratification is now based. Management of MB in adults is largely based on principles of managing children, modified according to differing clinical and toxicity profiles. Molecular analysis has led to the understanding that what was previously referred to as CNS-PNET comprise a heterogeneous group of tumours with a significant proportion representing other histologies. Atypical teratoid/rhabdoid tumour (AT/RT) carries mutations in the gene hSNF5/INI1 in most cases, with many now associated with an improved prospect of long-term survival. Pineal tumours have similar clinical presentations, comprising a heterogeneous mix of histologies from pineocytoma through to pineoblastoma.

Access to the complete content on Oxford Medicine Online requires a subscription or purchase. Public users are able to search the site and view the abstracts for each book and chapter without a subscription.

Please subscribe or login to access full text content.

If you have purchased a print title that contains an access token, please see the token for information about how to register your code.

For questions on access or troubleshooting, please check our FAQs, and if you can't find the answer there, please contact us.