Brain tumours marsh 2017

CNS TumorsCNS Tumors
 Brain TumorsTumors
 Prof. Nabil KhalilProf. Nabil Khalil

Core Learning Issues:
 Pathology +Major CLINICAL manifestations:
 Raised ICP – Pathology & Clinical features.
 Pathology of common Primary and secondary Brain tumors in
different age groups.
 Clinical presentations.

CNS Tumors: General Features
 10% of all tumors.
 Commonest solid cancers in children.(2nd
to Leuk for
all malignancies)
 Age: double peak 1st
& 6th
decade
 Adults - 70% supratentorial
 Children - 70% infratentorial
 No/very rare extraneural
spread.
 Metastasis most common.
AdultsAdults
Children
Children

Clinical features:
 Raised Intracranial Pressure*
Headache (morning), vomiting, slow pulse,
papilloedema.
 Local damage:
Lobes of the brain,Cerebellum,Nerve &
tract deficits.Symptoms of Paralysis,
seizures etc.

CNS Tum: Clinical Features-Pathogenesis
 Headaches (morning)
 Papilloedema
 Nausea or vomiting
 Bradycardia
 Seizures (convulsions).
 Drowsiness, Obtundation
 Personality or memory
 Changes in speech
 Limb weakness
 Balance/Stumbling
 eye movements or vision
 Increased ICP
 Increased ICP
 ICP – Medulla ob.
 ICP – Parasymp.
 Irritation.Cortex
 Brain Stem compress
 Frontal lobe
 Temporal lobe
 Motor area
 Cerebellum
 Optic tract, occipital.

CNS Anatomy - Clinical Features

Clinical symptoms
 Some Clinical symptoms

7th
nerve palsy:
 Cerebellopontine angle
tumours.
Acoustic neuroma,
epidermoid cysts,
medulloblastoma
meningioma
 Affected cranial nerves:
5 trigeminal - masticatio
7 facial –face muscles
8 auditory – hearing

Normal Fundus - Papilledema

Normal vs
Glaucoma

CNS Tumors Classification:
Primary Tumors:
Meninges – Meningioma
Glial cells: Glioma
Astrocytoma & Glioblastoma. Oligodendroma,
ependymoma.
Nerve sheath – Schwanoma, Neurofibroma.
Embryonal –PNET: Medulloblastoma,
neuroblastoma, teratoma.
Blood vessels – angioma, angiosarcoma etc.
* Other Epithelial, Pituitary (craniopharyngioma) &
Pineal gland tumors (pinloplastoma).
Secondary Tumors - Metastasis – common
Melanoma, breast, lung, GIT.

 Adults:
 Astrocytoma &
Glioblastoma.
 Meningioma
 Metastasis.
 Children:
 Astrocytoma
 Medulloblastoma
Common:

CNS Tumors: Summary
Adults:Adults:
 Secondaries common fromSecondaries common from
Lung, Skin melanomas,Lung, Skin melanomas,
breast..breast..
 Primary - SupratentorialPrimary - Supratentorial
 Astrocytoma /Astrocytoma /
glioblastoma.glioblastoma.
 MeningiomaMeningioma
Children:Children:
 22ndnd
common (leuk / lymph)common (leuk / lymph)
 InfratentorialInfratentorial
 Astrocytoma (cysticAstrocytoma (cystic
cerebellar)cerebellar)
 MedulloblastomaMedulloblastoma
 Hydrocephalus.Hydrocephalus.
 Meningeal spread.Meningeal spread.

Meningioma:
 Arise from arachnoid granulations of venous
sinuses. Attached to dura.
 Common sites: parasagittal (falx), sphenoid
ridge, olfactory groove, cerebellopontine
angle.  specific clinical features.
 Females common (2:1)
 Slow growth, well differentiated &
demarcated. Does not invade brain (Benign).
 Reactive skull Hyperostosis over the tumor.
 Microscopy: spindle cells in whorls and
psammoma bodies(microcalcification).

Meningioma—bilateral parasagittal-falx

Meningioma

Meningioma-ant. Falx+post

Meningioma ant .parasagittal

Meningioma-cortical

Meningioma-basal subfrontal
•Well demarcated
•Capsulated

Meningioma – whorls of clear cells.
Normal
Arachnoid
Granulation

Meningioma NodulesNodules
Psammoma Body

Glioma:
 Gliomas are neoplasms of glial cells.
 Commonest both in adults and
children
 Benign * to Aggressively malignant.
 Astrocytoma (anaplastic & G.B.M)
 Ependymoma - Rare, 4th ventricle.
 Oligodendroglioma - Benign, adults,
rare

Astrocytoma
s
Adults:
Commonest 80%, Supratentorial.
Solid – Fibrillary – low grade*.
Varigated, Hemorrhagic - Malignant,
glioblastoma multiforme.
Children:
Infratentorial (Cerebellum),
Cystic, Low grade*, Pilocytic

Astrocytoma-Lowgrade fibrillary

Astrocytoma

Astrocytoma: * Lat. Vent. *petechial hem.

Glioma Brain Stem – note diffuse tumor

Glioma Cerebrum cystic degeneration

Glioma:fronto-pareital

Astrocytoma (Glioma)cerebellar

Glioma Brain Normal

Glioblastoma Multiforme (GBM):
 High grade Astrocytoma - Grade IV
 Commonest & malignant brain tumor in adults –
mean survival <1y – cerebral supratentorial.
 Loss of heterozygosity on Chromosome 10 (80%)
 Most GBMs have lost one entire copy of C – 10
 2 types: Primary (worst) or Secondary from low grade
astrocytomas (better prog).
 Variants: giant cell GBM, gliosarcoma
 Microscopy:
 Necrosis, palisading, hypercellularity, nuclear atypia
& vascular proliferation & mitoses.

Genetic abnormalities in Glioma:
Low grade  Anaplastic  GBM

Glioma: high grade

Glioma:
Enhancement
with
peritumoral
edema.

Glioblastoma:

GBM:
+ glioma
Enhancement
with peritumoral
edema.

Glioblastoma – high grade Astrocytoma

Glioblastoma Multiforme (high grade Astrocytoma)

Glioblastoma Cerebrum-intraventricular

Compare
A Astrocytoma Low grade
B Glioblastoma Multiforme(GBM)
C Necrosis with
pseudopalisading in GBM.

Glioblastoma Multiforme
Palisading
Palisading
Necrosis
Necrosis

NecrosisNecrosis
PalisadingPalisading

Palisading
B.V
Necrosis

PalisadingNecrosis

Astrocytomas---Pilocytic:
Adults:
Commonest 80%, Supratentorial.
Solid – Fibrillary – low grade*.
Varigated, Hemorrhagic - Malignant,
glioblastoma multiforme.
Children:
Infratentorial (Cerebellum),
Cystic, Low grade*, Pilocytic

Pilocytic astrocytoma
 Common in childhood
 Most slow growing of the gliomas
 Sites: cerebellum, around 3rd
Ventricle. optic
nerve.
 Grossly cystic with mural nodule
 Microscopic
elongated hair-like (pilocytic) elongated
cells & Rosenthal fibers.

Pilocytic Astrocytoma - children

Pilocytic astrocytoma
Mural nodule

Pilocytic Astrocytoma: Microscopy
Palisading pilocytic astrocytes – note plenty of Rosenthal fibres between cells.

OLIGODENDROGLIOMA

Ependymoma

Ependymoma 4th
Ventricle

Ependymoma-hemorrhage

Primitive Neuroectodermal Tumors-PNET
 Origin from primitive blast cells.
Rosettes - attempted nerve formation.
 CNS
1. Medulloblastoma – Cerebellum
2. Pineal tumors(pinloplastoma)
Non CNS
1. Retinoblastoma – Retina(eye)
2. Neuroblastoma – Adrenal glands
3. Ganglioneuroma - Mediastinum

Medulloblastoma:
 Children.
 Cerebellum – vermis.
 Primitive neuroectodermal tum.PNET
 Blast cells – round scanty cytoplasm.
 4th
ventricle Obstruction – hydrocephalus.
 CSF seeding and Meningeal infiltration is
common.
 Rosettes & neuronal or glial differentiation
rarely seen.

Medulloblastoma:
Primitive neuroectodermal tumor:
Children, vermis of cerebellum.
Origin
Spread

Medulloblastoma

Epithelial tumours

Craniopharyngioma

pituitary tumors
 Pituitary adenomas:Tumors from anterior
pituitary component.Secreting or
nonSecreting chromopobe adenomas.
 Posterior pituitary (neuro-hypophysis):tumors
that decrease secreting Anti diuretic
hormone (ADH). Polyuria

Nerve Sheath Tumors

Nerve Sheath Tumors:
 Neurofibroma:
 Epi & endoneurial fibroblasts.
 Form whorls of fibroblasts with nerves
 Well differentiated, benign, capsulated.
 Schwannoma:
 Schwann cells, elongated form whorls
 Nuclear palisading

Schwannoma / Neurofibroma

Schwannoma 8th
Nerve:

Bilateral 8th
nerve schwannomas.

Schwannoma:

Schwannoma

Neurofibromatosis:

Neurofibromatosis:
Café-au-lait spot

32y Female with Fleshy pappules:

Neurofibromatosis:
 Autosomal dominant,
 NF1- Peripheral/Von Recklinghausen’s
 NF2- known as central NF.
 However, NF1 may cause central characteristics.
 About 50% familial, 50% sporadic gene mutation.
 NF1/ von Recklinghausen disease, gene mutation
on chromosome 17, 1 in every 3000-4000 births.
Diagnosis of NF1 if > 2 of
 6 or more café au lait spots (irregularly shaped, evenly
pigmented, brown macules),
 2 or more neurofibromas,
 axillary or inguinal freckling,
 Lisch nodules on the iris or optic glioma,
 various types of osseous lesions,
 a first-degree relative with the condition.

Neurofibromatosis:
 NF2 – Gene mutation chromosome 22.
 1 in every 33,000-40,000 births
 Typically present with acoustic neuromas or vestibular
schwannomas.
 Tinnitus, balance disorders, and progressive hearing loss
 May also have meningiomas and juvenile cataracts.
 First-degree relative and on any 2 of the conditions listed for NF1.
 Patients with NF1 are at increased risk of malignancy.
 Annual ocular examinations are recommended. Genetic testing is
also advocated in patients with NF who wish to have children.
 Surgery has been a successful treatment for the lesions
themselves; however, recurrence often occurs, and nerve
damage is a risk when tumors are located along neural pathways
 (National Institute of Neurologic Disorders and Stroke, 2006).

METASTASES
common brain tumours in adults especially in
the elderly

Metastatic Melanoma: multiple

Brain Metastases: Surrounding edema.

Most common CNS Tumors:
Glioblastoma MF

Summary:
 Children – 70% INFRAtentorial
 Adults – 70% SUPRAtentorial
 Common Malignant - adults, metastatic tumors (Lungs)
 Common - adults – glioblastoma multiforme
Intracerebral
 Common Benign - children – cerebellar astrocytoma.
 Common Malig - children – cerebellar
medulloblastoma
 Very rare in children– meninges and schwann cells
tumours(meningiomas and schwannomas) – usually
found in adults

Pathology ofPathology of
Increased Intracranial PressureIncreased Intracranial Pressure

Pathogenesis:
 Increased intracranial pressure (ICP): - if >
40 mm Hg  cerebral hypoxia, cerebral
ischemia, cerebral edema, hydrocephalus, and
brain herniation.
 Cerebral edema: Edema - Disruption of the
blood brain barrier – vasodilatation – swelling.
 Hydrocephalus  communicating OR NON
communicating type according to tumor site.

Pathogenesis:
 Brain herniation: Supratentorial herniation common.
3 sub types
 Subfalcine herniation: The cingulate gyrus of the frontal
lobe (commonest)
 Central transtentorial herniation: displacement of the
basal nuclei and cerebral hemispheres downward
 Uncal herniation: Medial edge of the uncus and the
hippocampal gyrus
 Cerebellar herniation: infratentorial herniation -
tonsil of the cerebellum is pushed through the
foramen magnum and compresses the medulla,
leading to bradycardia and respiratory arrest.

Common CNS Herniations:
 Subfalcine:

Subfalcine Herniation: in brain trauma.
Contusion of the inferior temporal
lobe (blue arrow) has resulted in
diffuse edema. (compressed and
flattened gyri on the right).
This has resulted in subfalcine
herniation of the cingulate gyrus
(red arrow), with a secondary
hemorrhagic infarction above that
(black arrow). A midline shift from
right to left is also present, as is
uncal herniation (yellow arrow).

Uncal Herniation:
Inferior view, The
herniated uncus is
bulging over the position
of the tentorium (black
arrows) and
compressing the
midbrain. The two
mammillary bodies (blue
arrows) have been
shifted to the patients
right due to the
pressure.

Uncal Herniation:

acute brain swelling-uncal herniation
 Swelling of the left
cerebral hemisphere
has produced a shift
with herniation of the
uncus of the
hippocampus through
the tentorium, leading
to the groove seen at
the white arrow.

Cerebellar Tonsil - Herniation
 Note the cone shape of the
herniated tonsils around the
medulla in this cerebellum
specimen.
 Results in compression and
hemorrhages in the pons.

Transtentorial herniation:
 Transtentorial herniation
at the base of the brain. A
prominent groove
surrounds the displaced
parahippocampal gyrus
(arrow). The adjacent 3rd
nerve (N) is compressed
and distorted and the
ipsilateral cerebral
peduncle (P) is distorted
with small areas of
haemorrhage.

Cerebral Herniation: Pathogenesis
Site ofSite of
herniationherniation EffectEffect Clinical consequenceClinical consequence
Transtentorial Ipsilateral 3rd cranial nerve
compression
Ipsilateral fixed dilated pupil
Ipsilateral 6th cranial nerve
compression
Horizontal diplopia, convergent
squint
Posterior cerebral artery
compression
Occipital infarction Cortical blindness
Cerebral peduncle
compression
Upper motor neurone signs
Brainstem compression and
haemorrhage
Decerebrate posture
Cardiorespiratory failure
Death
Foramen
magnum
Brainstem compression and
haemorrhage
Decerebrate posture
Cardiorespiratory failure Death
Acute obstruction of CSF
pathway
Decerebrate posture
Cardiorespiratory failure
Death

Decorticate posturing
Decorticate posturing, with elbows, wrists and
fingers flexed, and legs extended and rotated
inward.

Look for good in others…
no one is without faults and
everyone has some good
qualities!

52y, F, CNS tumor: ? diagnosis
1 2 3 4 5
0%
10%
90%
0%0%
1.1. Glioblastoma m.Glioblastoma m.
2.2. AstrocytomaAstrocytoma
3.3. MetastasesMetastases
4.4. MedulloblastomaMedulloblastoma
5.5. MeningiomaMeningioma

1 2 3 4 5
4%
90%
6%
0%0%
A.A. Glioblastoma m.Glioblastoma m.
B.B. AstrocytomaAstrocytoma
C.C. MeningiomaMeningioma
D.D. EpendymomaEpendymoma
E.E. MedulloblastomaMedulloblastoma

1 2 3 4 5
95%
2% 2%0%0%

52y, F, parasagittal tum attached to falx: ? diagnosis
1 2 3 4 5
6% 8% 10%
4%
71%
4.4. EpendymomaEpendymoma

Q:Commonest primary CNS tumor in Adults ?
1 2 3 4 5
15%
73%
0%0%
12%

52y, F, CNS tumor: ? Arrow Feature
1 2 3 4 5
0%
98%
0%2%0%
1.1. Necrosis.Necrosis.
2.2. Psammoma bodyPsammoma body
3.3. CalcificationCalcification
4.4. Blood vesselBlood vessel
5.5. Epithelial pearlEpithelial pearl

60y smoker, chronic bronchitis complains of
difficulty walking. , stiff, expressionless face. A
tremor of his fingers is apparent but ceases when
he tries to reach for something. Image shows
basal ganglia atrophy. Diagnosis?
1 2 3 4 5
0%
4% 2%
92%
2%
1.1. Alzheimers diseaseAlzheimers disease
2.2. Lacunar infarctsLacunar infarcts
3.3. Picks diseasePicks disease
4.4. Parkinsons diseaseParkinsons disease
5.5. haemorhagehaemorhage

Commonest primary CNS tumor in Children?
1 2 3 4 5
0%
67%
28%
4%
0%

Commonest Location of CNS tumor in Children?
1 2 3 4 5
4%
37%
4%
2%
53%
A.A. SupratentorialSupratentorial
B.B. CerebellumCerebellum
C.C. InfratentorialInfratentorial
D.D. Cerebrum.Cerebrum.
E.E. Brain stemBrain stem

astrocytoma

56y, F Rapidly growing parietal lobe tumor:? diagnosis
1 2 3 4 5
88%
0%
6%6%
0%

49y, M, CNS tumor: ? diagnosis
1 2 3 4 5
0% 0%
20%
80%
0%
A.A. MetastasesMetastases
B.B. Astrocytoma sy.Astrocytoma sy.
C.C. MeningiomatosisMeningiomatosis
D.D. NeurofibromatosisNeurofibromatosis
E.E. LipomatosisLipomatosis

SAQ / KFP
 Should seizure patients have
imaging done immediately?
 Personality changes indicate
which location?
 Differential diagnosis for young
adult with insidious symptoms,
seizures and decreased signal
on T1 and increased signal on
T2 weighted MRI?
 What is the treatment and
prognosis for someone with a
low-grade astrocytoma?
 How should the symptoms be
treated?
 Yes, 10-20% tumors.
 Frontal lobe
 Gliomas
 Conservative – Poor
 Steroids, anti
epileptic,
symptomatic.

SAQ / KFP
 Indication of a child hitting
his head?
 Why did the child have a
headache?
 If the child does have
hydrocephalus, at what level
is the ventricular system
being obstructed at?
 Should a lumbar puncture be
performed?
 Where in the cerebellum is
the lesion located?
 Indicating headache.
 Increased ICP, tum.
 4th
ventricle.
 No – coning…*
 Central – vermis

35y Male, depression
2-year history of loss of initiative, depression. He had
slowly lost his drive to win all the big deals he always
done so well at work. 3 months ago he began to
experience headache, which did not respond to
acetaminophen or aspirin. His wife noticed that his
lethargic state had increased in the past few months. 3
days ago his right arm began to convulse uncontrollably
for 1 minute. 1 day ago the patient began again violently
shaking his right arm, and the right side of face began to
twitch at the dinner table. No fever.
Physical exam: Bilateral papilledema, increased deep
tendon reflexes of the right bicep, tricep, +ve babinski
sign on the right foot, reduced leg strength on the right.

Axial T1 weighted MRI
Axial T2 weighted MRI

Coronal T1 weighted MRI

ASTROCYTOMA

3y Male, constant cry….
Constant crying and not interacting with other
children at daycare since 1m. Mother noticed
that he was pointing to his head often. Family
physician who stated that he was developing
normally, and that the “ terrible two’s” are difficult
period for parents. Recently started vomiting on
a daily basis and started wobbling even though
he learned to walk 6 months ago.
Physical: Bilateral papilledema and gait ataxia
was noted on the physical exam.

Axial T1 weighted MRI Axial T2 weighted MRI

4.4. EpendymomaEpendymoma
What is the most likely diagnosis?

THANK YOU

Brain tumours marsh 2017

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