Brain tumors

presented by
Dr. Suleiman Daoud
2021 WHO classification of the
tumors of CNS
Gliomas, glioneuronal tumors, and neuronal tumors
Adult-type diffuse gliomas Astrocytoma,
IDH-mutant Oligodendroglioma,
IDH-mutant, and 1p/19q-codeleted
GlioblastomaIDH-wildtype
Pediatric-type diffuse low-grade gliomas
Diffuse astrocytoma, MYB- or MYBL1-altered
Angiocentric glioma
Polymorphous low-grade neuroepithelial tumor of the young
Diffuse low-grade glioma, MAPK pathway-altered
Pediatric-type diffuse high-grade gliomas
Diffuse midline glioma H3 K27-altered
Diffuse hemispheric glioma H3 G34-mutant
Diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtype
Infant-type hemispheric glioma
Circumscribed astrocytic gliomas
Pilocytic astrocytoma
High-grade astrocytoma with piloid features
Pleomorphic xanthoastrocytoma
Subependymal giant cell astrocytoma
Chordoid glioma
Astroblastoma, MN1-altered
Glioneuronal and neuronal tumors
Ganglioglioma
Desmoplastic infantile ganglioglioma / desmoplastic infantile astrocytoma
Dysembryoplastic neuroepithelial tumor Diffuse glioneuronal tumor with
oligodendroglioma-like features and nuclear clusters
Papillary glioneuronal tumor
Rosette-forming glioneuronal tumor
Myxoid glioneuronal tumor
Diffuse leptomeningeal glioneuronal tumor
Gangliocytoma
Multinodular and vacuolating neuronal tumor
Dysplastic cerebellar gangliocytoma (Lhermitte-Duclos disease)
Central neurocytoma
Extraventricular neurocytoma
Cerebellar liponeurocytoma
Ependymal tumors
Supratentorial ependymoma
Supratentorial ependymoma, ZFTA fusion-positive
Supratentorial ependymoma, YAP1 fusion-positive
Posterior fossa ependymoma
Posterior fossa ependymoma, group PFA
Posterior fossa ependymoma, group PFB
Spinal ependymoma
Spinal ependymoma, MYCN-amplified
Myxopapillary ependymoma
Subependymoma
Choroid plexus tumors
Choroid plexus papilloma
Atypical choroid plexus papilloma
Choroid plexus carcinoma
Embryonal tumors

Medulloblastoma

Medulloblastomas molecularly defined

Medulloblastoma,WNT-activated
Medulloblastoma, SHH-activated and TP53-wildtype
Medulloblastoma, SHH-activated and TP53-mutant
Medulloblastoma, non-WNT/non-SHH

Medulloblastomas, histologically defined

CNS WHO grading of selected
Types
Astrocytoma, IDH-mutant 2, 3, 4
Oligodendroglioma, IDH-mutant, and 1p/19q-codeleted 2, 3
Glioblastoma, IDH-wildtype 4
Diffuse astrocytoma, MYB- or MYBL1-altered 1
Polymorphous low-grade neuroepithelial tumor of the young 1
Diffuse hemispheric glioma, H3 G34-mutant 4
Pleomorphic xanthoastrocytoma 2, 3
Multinodular and vacuolating neuronal tumor 1
Supratentorial ependymoma 2, 3
Posterior fossa ependymoma 2, 3
Myxopapillary ependymoma 2
Meningioma 1, 2,
3
Solitary fibrous tumor
1,2,3
NOS (Not Otherwise Specified)
Adding an NOS suffix indicates that the diagnostic information
(histological or molecular) necessary to assign a specific WHO
diagnosis is not available, providing an alert to the oncologist that a
molecular work-up has not been undertaken or failed technically

NEC (Not Elsewhere Classified)

An NEC suffix, on the other hand, indicates that the necessary
diagnostic testing has been successfully performed but that the
results do not readily allow for a WHO diagnosis; for example, if
there is a mismatch between clinical, histological,
immunohistochemical, and/or genetic features
Layered Report Structure
Integrated diagnosis (combined tissue-
based histological and molecular
diagnosis) Histological diagnosis CNS
WHO grade Molecular information
Newly Recognized Tumor Types in the 2021 WHO
Classification of Tumors of the Central Nervous
System
Diffuse astrocytoma, MYB- or MYBL1-altered
Polymorphous low-grade neuroepithelial tumor of the young
Diffuse low-grade glioma, MAPK pathway-altered
Diffuse hemispheric glioma, H3 G34-mutant
Diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtype Infant-type
hemispheric glioma High-grade astrocytoma with piloid features Diffuse glioneuronal
tumor with oligodendroglioma-like features and nuclear clusters (provisional type)
Myxoid glioneuronal tumor
Multinodular and vacuolating neuronal tumor
Supratentorial ependymoma, YAP1 fusion-positive
Posterior fossa ependymoma, group PFA
Posterior fossa ependymoma, group PFB
Spinal ependymoma, MYCN-amplified Cribriform neuroepithelial tumor (provisional type)
CNS neuroblastoma, FOXR2-activated
CNS tumor with BCOR internal tandem duplication
Desmoplastic myxoid tumor of the pineal region, SMARCB1-mutant
Intracranial mesenchymal tumor, FET-CREB fusion positive (provisional type)
CIC-rearranged sarcoma
Primary intracranial sarcoma, DICER1-mutant
Pituitary blastoma
Division of diffuse gliomas into adult-type and pediatric
type.
Simplification of the classification of common,
adulttype, diffuse gliomas.
CNS classification from 2016, the common diffuse gliomas of adults were
divided into 15 entities.

WHO CNS5, on the other hand, includes only 3 types:

Astrocytoma, IDH-mutant; Oligodendroglioma, IDH-
mutant and 1p/19q-codeleted; and Glioblastoma, IDH-
wildtype.
Pediatric-type low-grade and high-grade diffuse
gliomas
Basic information about Brain
tumors
Primary vs secondary.
Intra-axial vs Extra-axial .
CT Brain w and w/o contrast.
MRI Brain w and w/o contrast with other
sequences ex: FLAIR, DIFFUSION ….
MANIFESTATION OF BRAIN
TUMORS
SUPRA-TENTORIAL :
H/A. In addition to other high ICP
symptoms and signs
PROGRESSIVE FOCAL DEFICITS.
SEZIURE.
CVA LIKE PICTURE….HMG OR
EDEMA.
PERSONALITY CHANGES.
INFRA-TENTORIAL
HIGH ICP SIGNS: TUMOR ITSELF
AND ASSOCIATED HCP
H/A.
NAUSEA AND VOMITING.
ATAXIA AND OTHER CEREBELLAR
SIGNS
GLIOMA

CLINICO-PATHOLOGICAL AND
MOLECULAR INTERGRATED
DIAGNOSIS.
MOST COMMON IS GRADE 4 : GBM.
MRI BRAIN W CONTRAST AND
FLAIR/T2 SEQUENCE ARE
ESSENTIAL.
CT SCAN ….LOOKING FOR HMG
AND CALCIFICATION.
GBM

GLIOBLASTOMA IDH- WILDTYPE.

RING ENHANCING LESION WITH
CONTRAST.
MOST COMMON PRIMARY GLIAL
TUMOR
DDX OF RING ENHANCING LESIONS:
GBM
BRAIN ABCESS.
METASTASIS.
PRIMARY CNS LYMPHOMA.
SUBACUTE STROKE.
RESOLVING ICH
NEUROCYSTICERCOSIS.
DEMYELINATION ( INCOMPLETE RING).
Pilocystic astrocutoma

CIRCUMSCIBED ASTROCYTIC
GLIOMA.
1ST AND 2ND DECADE .
INFRA-TENTORIAL
CYSTIC LESION WITH MURAL
NODULE .
HAS EXCELLENT PROGNOSIS IF
COMPLETELY RESECTED.
Subependymal giant cell astrocytomas
CIRCUMSCRIBED ASTROCYTIC
GLIOMA .
TUBEROUS SCLEROSIS
OPTIC GLIOMA:
NF1 VS SPORADIC .
MENINGIOMA
Meningiomas account for 15–20% of all
intracranial neoplasms, second only to the
incidence of primary gliomas.
M:F 1:2 .
Intracranial distribution of meningiomas :
25% parasagittal.
19% convexity.
17% sphenoid ridge.
9% suprasellar .
In children accounts about 1-4%.
M:F 1:1 .
More malignant.
Most common sites : intraventricuar and
posterior fossa .
Association with NF1
Multiple meningiomas manily seen in
NF2>NF1.
Malignant meningiomas seen in about
1-3 %.
Spinal meningiomas mainly intradural
extramedullary .
Most common site thoracic spine.

M:F 1:3-6 . EXCEPT lumbar spine

1:1.42
Origin from arachnoid cap cells.
Grading system :
Grade 1: meningiothelial, fibrous,
transitional ,psammomatous,
angiomatous ……etc.
Grade 2: atypical ,clear cell and
chordoid.
Grade 3: rhabdoid, papillary and
anaplastic.
Main blood supply from the external
carotid artery.
Olfactory groove
meningioma,intraventricular and
tentorial meningiomas…..from the
internal carotid branches.
Olfactory groove meningiomas
….anterior ethmoidal…..ophthalmic
artery…..(do not embolize
PRIMARY CNS LYMPHOMA
Non –hodgkin B –cell lymphoma.
Seen in increase frequency in
immunocomprised patients.
May involve the hemisphere,spinal cord
and eyes.
Affecting deep grey matter nuclei,
frontal lobe and midline structures.
CT brain usually hyperdense , multiple
lesions, enhanced homogeneously.
May resemble meningioma…..no
calcification.
Ghost tumor….disappears with
steroids.
MRI BRAIN in immunocompromised
patients …..ring enhancing….target
lesion.
STEREOTACTIC BIOPSY FOR
HAEMANIOBLASTOMA

1-2% of brain tumor.

Sporadic or linked to VON HIPPEL
LINDAU.
Cystic lesion with mural nodule…resemble
Pilocytic astrocytoma.
The most common primary intra-axial
posterior fossa tumor in adult.
Single or multiple lesions in the posterior
fossa in adult ….rule out metastasis first.
CEREBRAL METASTASIS
Most commonly found at the grey-white
matter junction and superficial distal arterial
fields.
80% cerebrum, 15% cerebellar and 5%
brain stem.
Adenocarcinoma of the breast and colon,
and renal cell and thyroid carcinomas tend
to produce a single metastasis, whereas
melanoma and lung cancer usually result in
multiple metastases.
Posterior fossa masses in
children
Posterior fossa astrocytoma
Pilocytic astrocytoma
Brainstem glioma
Medulloblastoma.
Ependymoma
Atypical teratoid/rhabdoid tumor (AT/RT)
Hemangioblastoma (uncommon except in
patients with vHL)
Teratoma (in infants)
LP CONTRA-INDICATED.
WHOLE SPINE MRI IS INDICATED
LOOKING FOR DROP METASTASIS:
MEDULLOBLASTOMA.
EPENDYMOMAS.
Choroid Plexus
Papilloma
* Rare
•From the Choroid
plexus 🡪 inc.CSF
production

🡪Hydrocephalus.
•Supratentorial 🡪
Children ( Lateral
Vent. )

Infratentorial 🡪 Adult
( Rare )
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