The Comparison between WHO

2007 and WHO 2016 Grading of

Tumours

Dr. Riski Amanda

11/15/2018 dr.riskiamanda@gmail.com 1
• 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, which should be
considered a replacement for the earlier
version.
11/15/2018 dr.riskiamanda@gmail.com 2
• 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.

11/15/2018 dr.riskiamanda@gmail.com 3
 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
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• 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.

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 Structure

• Despite a move towards molecular markers for some

entities, the classification continues to be organised
according to the cell of origin (e.g. ependymal
tumours) or anatomical origin (e.g. tumours of the
sellar region).
• For entities that now incorporate molecular markers
into their definition (e.g. oligodendroglioma IDH-
mutant, 1p19q co-deleted) it is recognised that such
markers are not always available or identified. In such
cases, a 'not otherwise specified (NOS)' option is
available (e.g. oligodendroglioma NOS).

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 Classification

• NOS: not otherwise specified

• 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

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 Diffuse astrocytic and
oligodendroglial tumours
• WHO grade II
– diffuse astrocytoma
• IDH-mutant - 9400/3
– gemistocytic astrocytoma - 9411/3
• IDH-wildtype - 9400/3
• NOS - 9400/3
– oligoastrocytoma NOS - 9382/3
– oligodendroglioma - 9450/3
• IDH-mutant, 1p19q co-deleted
• oligodendroglioma NOS

• WHO grade III

– anaplastic astrocytoma - 9401/3
• IDH-mutant
• IDH-wildtype
• NOS
– anaplastic oligoastrocytoma NOS - 9382/3
– anaplastic oligodendroglioma - 9451/3
• IDH-mutant, 1p19q co-deleted
• anaplastic oligodendroglioma NOS

• WHO grade IV
– glioblastoma
• IDH wildtype - 9440/3
– giant cell glioblastoma - 9441/3
– gliosarcoma 9442/3
– epithelioid glioblastoma - 9440/3
• IDH mutant - 9440/3 *
• NOS - 9440/3
• diffuse midline glioma, H3K27M-mutant *

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 Other astrocytic tumours

• WHO grade I
– pilocytic astrocytoma 9421/11 - WHO grade I
– subependymal giant cell astrocytoma 9384/1 - WHO grade
I

• WHO grade II
– pilomyxoid astrocytoma 9425/3
– pleomorphic xanthoastrocytoma 9424/3 - WHO grade II

• WHO grade III

– anaplastic pleomorphic xanthoastrocytoma 9424/3 - WHO
grade III

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 Astrocytic Tumours
Astrocytic tumours WHO 2007 WHO 2016
Grade Grade
I II III IV I II III IV
Subependymal giant cell astrocytoma ● ●
Pilocytic astrocytoma ● ●
Pilomyxoid astrocytoma ●
Diffuse astrocytoma ●
Pleomorphic xanthoastrocytoma ● ●
Anaplastic astrocytoma ● ●
Glioblastoma ●
Giant Cell Glioblastoma ●
Gliosarcoma ●
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 Oligodendroglial Tumours
Astrocytic tumours WHO 2007 WHO 2016
Grade Grade
I II III IV I II III IV
Oligodendroglioma ●
Anaplastic Oligodendroglioma ●

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 Additional
Diffuse Astrocytic and Oligodendroglial Tumours
Selected CNS Tumours WHO 2007 WHO 2016
Grade Grade
I II III IV I II III IV
Diffuse astrocytoma, IDH-mutant ●
Anaplastic astrocytoma, IDH-mutant ●
Glioblastoma- IDH-wildtype ●
Glioblastoma, IDH-mutant ●
Diffuse midline glioma, H3 K27M-mutant ●
Oligodendroglioma, IDH-mutant and 1p/19q- ●
codeleted
Anaplastic oligodendroglioma, IDH mutant ●
and 1p/19q-codeleted
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 Oligoastrocytic Tumours
WHO007 WHO 2016
Grade Grade
I II III IV I II III IV
Oligoastrocytoma ●
Anaplastic oligoastrocytoma ●

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 Additional

Other Astrocytic Tumours

Selected CNS Tumours WHO 2007 WHO 2016
Grade Grade
I II III IV I II III IV
Pilocytic Astrocytoma ●
Subependymal Giant Cell Astrocytoma ●
Pleomorphic Xanthoastrocytoma ●
Anaplastic Pleomorphic Xanthoastrocytoma ●

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 Ependymal tumours

• WHO grade I
– subependymoma - 9383/1
– myxopapillary ependymoma - 9394/1

• WHO grade II
– ependymoma - 9391/3
• papillary ependymoma
• clear cell ependymoma
• tanycytic ependymoma
• RELA fusion-positive - 9396/3 *

• WHO grade III

– anaplastic ependymoma - 9392/3

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 Ependymal Tumours
WHO007 WHO 2016
Grade Grade
I II III IV I II III IV
Subependymoma ● ●
Myxopapilary Ependymoma ● ●
Ependymoma ● ●
Anaplastic Ependymoma ● ●

ADDITIONAL
Ependymoma, RELA fusion-positive ● ●

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 Other gliomas

• WHO grade I
– angiocentric glioma - 9431/1

• WHO grade II
– chordoid glioma of the third ventricle - 9444/1

• WHO grade not yet assigned

– astroblastoma - 9430/3

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 Additional

Other Gliomas
Selected CNS Tumours WHO 2007 WHO 2016
Grade Grade
I II III IV I II III IV
Angiocentric Glioma ●
Chordoid glioma of third ventricle ●

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 Choroid plexus tumours

• WHO grade I
– choroid plexus papilloma - 9390/0

• WHO grade II
– atypical choroid plexus papilloma - 9390/1

• WHO grade III

– choroid plexus carcinoma - 9390/3

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 Choroid Plexus Tumours
WHO007 WHO 2016
Grade Grade
I II III IV I II III IV
Choroid Plexus Papiloma ● ●
Atypical Choroid Plexus Papiloma ● ●
Choroid Plexus Carcinoma ● ●

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 Other Neuroepithelial Tumours
WHO007 WHO 2016
Grade Grade
I II III IV I II III IV
Angiocentric Glioma ● ●
Choroid Glioma of The Third Ventricle ● ●

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 Neuronal and mixed neuronal-glial
tumours
• WHO grade I
– desmoplastic infantile astrocytoma and ganglioglioma - 9412/1
– dysembryoplastic neuroepithelial tumour (DNET) - 9413/0
– dysplastic gangliocytoma of the cerebellum - (Lhermitte-Duclos) - 9493/0
– gangliocytoma - 9492/0
– ganglioglioma - 9505/1
– papillary glioneuronal tumour - 9509/1
– paraganglioma of the filum terminale - 8680/1
– rosette-forming glioneuronal tumour of the fourth ventricle - 9509/1

• WHO grade II
– central neurocytoma - 9506/1
– extraventricular neurocytoma - 9506/1
– cerebellar liponeurocytoma - 9506/1

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• WHO grade III
– anaplastic ganglioglioma - 9505/3

• WHO grade unknown

– diffuse leptomeningeal glioneuronal tumour - no
IDC-O code *

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 Neural and Mixed Neuronal-glial Tumours
WHO007 WHO 2016
Grade  I II III IV I II III IV
Gangliocytoma ● ●
Ganglioglioma ● ●
Anaplastic Ganglioglioma ● ●
Desmoplastic Infantile Astrocytoma and ● ●
Ganglioglioma
Dysembryoplastic Neuroepithelial tumour ● ●
Central Neurocytoma ● ●
Extraventricular Neurocytoma ● ●
Cerebellar Liponeurocytoma ● ●
Paragangliomaof the Spinal Cord ●
Papillary Glioneural Tumour ● ●
Rosette-forming Glioneural Tumour of the ● ●
fourth ventricle
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 Additional

Neural and Mixed Neuronal-glial Tumours

Selected CNS Tumours WHO 2007 WHO 2016
Grade Grade
I II III IV I II III IV
Dysplastic gangliocytoma of cerebellum ●
(Lhermitte-Ducios)

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 Tumours of the pineal region

• WHO grade I
– pineocytoma - 9361/1

• WHO grade II or III

– pineal parenchymal tumour of intermediate
differentiation - 9362/3
– papillary tumour of the pineal region - 9395/3

• WHO grade IV
– pineoblastoma - 9362/3

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 Pineal Tumours
WHO007 WHO 2016
Grade Grade
I II III IV I II III IV
Pineocytoma ● ●
Pineal parenchymal tumour of intermediate ● ● ● ●
differentiation
Pineoblastoma ● ●
Papilary tumour of the pineal region ● ● ● ●

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 Embryonal tumours
• WHO grade IV
– medulloblastoma
• genetically defined
– WNT-activated - 9475/3 *
– SHH-activated & TP53-mutant - 9476/3
– medulloblastoma SHH-activated & TP53-wildtype - 9471/3
– group 3 - 9477/3
– group 4 - 9477/3
• histologically defined
– classic - 9470/3
– desmoplastic/nodular - 9471/3
– extensive nodularity - 9471/3
– large cell/anaplastic - 9470/3
– NOS - 9470/3
• CNS neuroblastoma 9500/3
• CNS ganglioneuroblastoma 9490/3
• embryonal tumours with multilayered rosettes - 9478/3 *
– C19MC-altered
– NOS
• medulloepithelioma 9501/3
• atypical teratoid / rhabdoid tumour 9508/3
• CNS embryonal tumour with rhabdoid features 8508/3
• CNS embryonal tumour, NOS 9473/3
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 Embryonal Tumours
WHO007 WHO 2016
Grade Grade
I II III IV I II III IV
Medulloblastoma ●
CNS primitive neuroectodermal tumour ●
(PNET)
Atypical teratoid / rhabdoid tumour ●

ADDITIONAL
Embryonal tumour with multilayered rosettes, ●
C 19MC-altered
Medulloepithelioma ●
CNS embryonal tumour, NOS ●
CNS embryonal tumour with rhabdoid ●
features
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 Tumours of cranial and paraspinal
nerves
• WHO grade I
– schwannoma (neurilemoma, neurinoma) - 9560/0
• cellular schwannoma
• plexiform schwannoma
• melanotic schwannoma- 9560/1
– neurofibroma - 9540/0
• atypical neurofibroma - 9540/0
• plexiform neurofibroma - 9550/0
– perineurioma 9571/0

• WHO grade II, III or IV

– malignant peripheral nerve sheath tumour (MPNST) - 9540/3
• epithelioid
• with perineural differentiation

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 Tumours of the Cranial and Paraspinal Nerves

WHO007 WHO 2016

Grade Grade
I II III IV I II III IV
Schwannoma ● ●
Neurofibroma ● ●
Perineurioma ● ● ● ●
Malignant peripheral nerve sheath tumour ● ● ● ● ● ●
(MPNST)

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31z
 Tumours of meningothelial cells
• WHO grade I
– meningioma - 9530/0
– meningothelial meningioma - 9531/0
– fibrous meningioma - 9532/0
– microcystic meningioma - 9530/0
– psammomatous meningioma - 9533/0
– angiomatous meningioma - 9534/0
– secretory meningioma - 9530/0
– metaplastic meningioma - 9530/0
– lymphoplasmacyte-rich meningioma - 9530/0
• WHO grade II
– atypical meningioma - 9539/1
– clear cell meningioma - 9538/1
– chordoid meningioma - 9538/1

• WHO grade III

– anaplastic meningioma (malignant) - 9530/3
– papillary meningioma - 9538/3
– rhabdoid meningioma - 9538/3

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 Meningeal Tumours
WHO007 WHO 2016
Grade Grade
I II III IV I II III IV
Meningioma ● ●
Atypical meningioma ● ●
Anaplastic / malignant meningioma ● ●
Haemangiopericytoma ●
Anaplastic haemangiopericytoma ●
Haemangioblastoma ●

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 Mesenchymal, non-meningothelial tumours
• WHO grade I, II or III
– solitary fibrous tumour of the dura/haemangiopericytoma - 8815/0 /1
/3

• WHO grade I
– angiolipoma - 8861/0
– chondroma - 9220/0
– desmoid-type fibromatosis - 8821/1
– haemangioblastoma - 9161/1
– haemangioma - 9120/0
– hibernoma - 8880/0
– leiomyoma - 8890/0
– lipoma - 8850/0
– myofibroblastoma - 8825/0
– osteochondroma - 9210/0
– osteoma 9180/0
– rhabdomyoma - 8900/0

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• WHO grade III
• epithelioid haemangioendothelioma - 9133/3
• angiosarcoma - 9120/3
• chondrosarcoma - 9220/3
• Ewing sarcoma / PNET - 9364/3
• fibrosarcoma - 8810/3
• Kaposi sarcoma - 9140/3
• leiomyosarcoma - 8890/3
• liposarcoma (intracranial) - 8850/3
• osteosarcoma - 9180/3
• rhabdomyosarcoma - 8900/3
• undifferentiated pleomorphic sarcoma / malignant fibrous
histiocytoma - 8830/3

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 Additional

Mesenchymal, Non-Meningothelial Tumours

Selected CNS Tumours WHO 2007 WHO 2016
Grade Grade
I II III IV I II III IV
Solitary fibrous tumour / ● ● ●
haemangiopericytoma
Haemangioblastoma ●

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 Tumours of the sellar region

• WHO grade Icraniopharyngioma - 9350/1

– adamantinomatous - 9351/1
– papillary - 9352/1
• granular cell tumour - 9582/0
• pituicytoma - 9432/1
• spindle cell oncocytoma - 8291/0

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 Tumours of the Sellar Region
WHO007 WHO 2016
Grade Grade
I II III IV I II III IV
Craniopharyngioma ● ●
Granular cell tumour of the neurohypophysis ● ●
Pituicytoma ● ●
Spindle cell oncocytoma of the ● ●
adenohypophysis

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