Recent Edits
Log In
Articles
Sign Up
Cases
Courses
Quiz
Donate
About
ADVERTISEMENT: Radiopaedia is free thanks to our supporters and advertisers. Become a Gold Supporter and see no third-party ads.
Articles
Cases
Courses
REGISTER NOW

WHO classification of CNS tumours

Changed by Craig Hacking, 24 Jun 2016
Back to revision history

Updates to Article Attributes

Body was changed:

The WHO classification of CNS tumours is the most widely accepted system for classifying CNS tumours and was based on the histological characteristics of the tumour. Although the most recent version of the 'blue book' is the 4th edition from 2007, an update has been released in 2016 3, which should be considered a replacement of the earlier version. "At this point, a decision to undertake the 5th edition series of WHO Blue Books has not been made" 3. This 2016 update has, for the first time, included molecular parameters into the diagnostic schema, and in fact has elevated them in some instances above histological features 3.  

This is a fairly dry article, primarily for reference. For more readable versions, see the article on brain tumours and brain tumours in infancy for general discussion of these topics.

Main changes since previous (2007) version

The most recent update (2016) has significantly changed the classification of a number of tumour families, introducing a greater reliance on molecular markers. The most notable changes involve diffuse gliomas, in which IDH status (mutated vs wildtype) and 1p19q co-deletion (for oligodendrogliomas) have risen to prominence. Importantly if histological phenotype and genotype are not-concordant (e.g. looks like diffuse astrocytoma but is 1p19q co-deleted, ATRX-wildtype) then genotype wins and it is used to determine diagnosis 3. 

Medulloblastomas have also been divided into distinct molecular subgroups. 

Another change is the combining of solitary fibrous tumours of the dura with hemangiopericytoma, which although appearing very different on imaging seem now to be manifestations of the same tumour. 

Classification

Explanatory notes
  • four digit code: is from the International Classification of Disease for Oncology (ICD-O)
  • /: the number after the slash (/) refers to biological behaviour, not WHO Grade
  • *: refers to a 'new' tumour in the classification
  • italics: refers to a provisional inclusion
  • NOS: not otherwise specified 
Diffuse astrocytic and oligodendroglial tumours
Other astrocytic tumour
Ependymal tumours
Other gliomas
Choroid plexus tumours
Neuronal and mixed neuronal-glial tumours
Tumours of the pineal region
Embryonal tumours
Tumours of cranial and paraspinal nerves
Tumours of meningothelial cells
Mesenchymal, non-meningothelial tumours
Melanocytic lesions
Lymphomas
  • diffuse large B-cell lymphoma of the CNS - 9680/3
  • immunodeficiency-associated CNS lymphomas
    • AIDS-related diffuse large B-cell lymphoma
    • EBV-positive diffuse large B-cell lymphoma, NOS
    • lymphomatoid granulomatosis - 9766/1
  • intravascular large B-cell lymphoma - 9712/3
  • low grade B-cell lymphomas of the CNS
  • T-cell and NK/T-cell lymphomas of the CNS
  • anaplastic large cell lymphoma
    • ALK-positive - 9714/3
    • ALK-negative - 9702/3
  • MALT lymphoma of the dura - 9699/3
Histiocytic tumours
Germ cell tumours
Tumours of the sellar region

Entities removed

The following entities are no longer found in the 2016 WHO classification, last seen in the 2007 version. 

Although oligoastrocytomas remain entities they are now going to be rare, requiring molecularly distinct populations of both components: astrocytes (IDH-mutant, ATRX-mutant, 1p19q-intact) and oligodendroglial cells (IDH-mutant, ATRX-wildtype, 1p19q-co-deleted) 3. 

New entities

The following entities have been introduced in the 2016 WHO classification.

  • -<p>The <strong>WHO classification of CNS tumours</strong> is the most widely accepted system for classifying CNS tumours and was based on the <a href="/articles/who-grading-of-cns-tumours">histological characteristics</a> of the tumour. Although most recent version of the 'blue book' is the 4<sup>th </sup>edition from 2007, an update has been released in 2016 <sup>3</sup>, which should be considered a replacement of the earlier version. "At this point, a decision to undertake the 5<sup>th </sup>edition series of WHO Blue Books has not been made" <sup>3</sup>. This 2016 update has, for the first time, included molecular parameters into the diagnostic schema, and in fact has elevated them in some instances above histological features <sup>3</sup>.  </p><p>This is a fairly dry article, primarily for reference. For more readable versions, see the article on <a href="/articles/brain-tumours">brain tumours</a> and <a href="/articles/brain-tumours-in-infancy">brain tumours in infancy</a> for general discussion of these topics.</p><h4>Main changes since previous (2007) version</h4><p>The most recent update (2016) has significantly changed the classification of a number of tumour families, introducing a greater reliance on molecular markers. The most notable changes involve diffuse gliomas, in which <a href="/articles/isocitrate-dehydrogenase-idh">IDH</a> status (mutated vs wildtype) and 1p19q co-deletion (for oligodendrogliomas) have risen to prominence. Importantly if histological phenotype and genotype are not-concordant (e.g. looks like diffuse astrocytoma but is 1p19q co-deleted, ATRX-wildtype) then genotype wins and it is used to determine diagnosis <sup>3</sup>. </p><p>Medulloblastomas have also been divided into distinct molecular subgroups. </p><p>Another change is the combining of solitary fibrous tumours of the dura with hemangiopericytoma, which although appearing very different on imaging seem now to be manifestations of the same tumour. </p><h4>Classification</h4><h6>Explanatory notes</h6><ul>
  • +<p>The <strong>WHO classification of CNS tumours</strong> is the most widely accepted system for classifying CNS tumours and was based on the <a href="/articles/who-grading-of-cns-tumours">histological characteristics</a> of the tumour. Although the most recent version of the 'blue book' is the 4<sup>th </sup>edition from 2007, an update has been released in 2016 <sup>3</sup>, which should be considered a replacement of the earlier version. "At this point, a decision to undertake the 5<sup>th </sup>edition series of WHO Blue Books has not been made" <sup>3</sup>. This 2016 update has, for the first time, included molecular parameters into the diagnostic schema, and in fact has elevated them in some instances above histological features <sup>3</sup>.  </p><p>This is a fairly dry article, primarily for reference. For more readable versions, see the article on <a href="/articles/brain-tumours">brain tumours</a> and <a href="/articles/brain-tumours-in-infancy">brain tumours in infancy</a> for general discussion of these topics.</p><h4>Main changes since previous (2007) version</h4><p>The most recent update (2016) has significantly changed the classification of a number of tumour families, introducing a greater reliance on molecular markers. The most notable changes involve diffuse gliomas, in which <a href="/articles/isocitrate-dehydrogenase-idh">IDH</a> status (mutated vs wildtype) and 1p19q co-deletion (for oligodendrogliomas) have risen to prominence. Importantly if histological phenotype and genotype are not-concordant (e.g. looks like diffuse astrocytoma but is 1p19q co-deleted, ATRX-wildtype) then genotype wins and it is used to determine diagnosis <sup>3</sup>. </p><p>Medulloblastomas have also been divided into distinct molecular subgroups. </p><p>Another change is the combining of solitary fibrous tumours of the dura with hemangiopericytoma, which although appearing very different on imaging seem now to be manifestations of the same tumour. </p><h4>Classification</h4><h6>Explanatory notes</h6><ul>

Systems changed:

  • Spine

ADVERTISEMENT: Supporters see fewer/no ads

Articles

By Section:

By System:

Cases

Radiopaedia.org

Contact Us
Terms of Use
Privacy Policy
Licensing
Sponsorship
Developers

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.

© 2005–2024 Radiopaedia.org