Tumours of The Central Nervous System: FM Brett MD., Frcpath

TUMOURS OF THE CENTRAL
NERVOUS SYSTEM
FM Brett MD., FRCPath
At the end of this lecture you

should be able to:
1. Give basic classification of CNS tumours
2. Understand how patients present
3. Know the common tumours in children and adults
4. Know what is meant by paraneoplastic syndromes
5. Know that concept of benign and malignant
meaningless when applied to CNS tumours
CLASSIFICATION OF CNS
TUMOURS
1. Intrinsic tumours account for virtually all
tumours in children and 60% of primary CNS
tumours in adults
2. Extrinsic tumours arising from cranial and

spinal nerves and dura.
3. Tumours arising from adjacent structures i.e
pituitary gland and metastatic tumours.
The pathologist and CNS

neoplasms
Clinical details of importance
~
~
~
~
Age
Sex
F/X
Site of neoplasm
INCIDENCE;
~ Second commonest form of cancer in children
Accounts for 3.5% of all deaths in the 1-14
year age group
Sixth commonest cause of cancer deaths in adults
25% of all tumors in adults are in the brain and 35%
are neurectodermal and 40% are metastatic
~ Most primary tumors are sporadic and

of unknown aetiology
~ Secondary tumors vary greatly
between 14-40%
~ Fewer than 5% are associated with
hereditary syndromes that predispose
to neoplasia
Heritable syndromes with increased risk of CNS tumours

Syndrome
Gene locus
Gene
Type of CNS tumour
NF type 1
17q11
NF1
Neurofibromameningio
ma, optic nerve glioma
NF2
22q12
NF2
Meningioma,
schwannoma
TS
9q34,16p13
TSc1/TSC2
SEGA
VHL
3p35
VHL
Haemangioblastoma
LiFraumani
17q13
p53
glioma
Gorlins
syndrome
9q31
PNET
CNS neoplasms present with:

~ epilepsy (focal or generalised)
~ focal neurologic deficits
~ symptoms and signs of raised ICP
~ symptoms and signs of hydrocephalus
Sites of cerebral tumors

SSites of cerebral tumors
ADULTS
Supratentorial tumors account for 90%
Therefore increased incidence of epilepsy
and decreased incidence of headache
Posterior fossa tumours cause headache
and vomiting as early features
CHILDREN
Cerebellum
Pons
Optic nerve/chiasm
SUPRATENTORIAL TUMORS ARE
RARE
Therefore
Headache, vomiting, visual disturbances
common
Epilepsy - unusual
Diagnosis
1. Clinical picture
2. CT or MRI scan
3. Biopsy
~ smear
~ Frozen section
~ paraffin section
Factors in the aetiology of CNS neoplasms
1. Sex gliomas commoner in males

meningiomas commoner in females
2. Exposure to ionizing radiation implicated
in the genesis of
~ meningiomas
~ gliomas
~ nerve sheath tumors
3. Primary CNS lymphoma is associated with
immunodeficiency
4. Nitroso compounds cause CNS neoplasms in animals
5. No convincing evidence has linked CNS neoplasms
with trauma, occupation, diet, electromagnetic fields
Prognostic factors in CNS tumors

~ Patient characteristics
~ Tumour characteristics
Patient characteristics
~ Age
~ General physical characteristics
~ Extent of surgical resection
Tumour characteristics
~ Specimen procurement
~ Phenotypic analysis
~ Proliferative capacity
EFFECTS OF TUMOUR
1.
2.
3.
4.
Local destruction of neural tissue

Oedema
Distortion of neural tissue
Raised ICP
Grading of Gliomas (WHO)

Grade 1 1V based on presence of pleomorphism,
mitoses, vascular proliferation and necrosis
Median Overall Survival AA 3-5 years
OS GBM 1 year
Secondary GBM younger patients with pre-existing
lower grade glioma
Primary 60-70
Primary GBM
high frequency of RGFR amplification
-p16 loss
-Secondary GBM TP53 mutations
Oligodendroglioma
~ Concurrent deletion of 1p and 19q
In AO good response to DXT and chemo
~ Criteria for anaplasia nuclear pleomorphism,
mitotic activity, endothelial vascular hyperplasia
and necrosis
~ For anaplasia 2 features one of which frequent
mitoses or VEH
Predictive Markers in Malignant Gliomas

~ 1p19q loss in AO associated with
enhanced chemosensitivity and longer
overall survival
~ MGMT status in GBM inc responsivness to
temezolamide
~ EGFR inc in GBM
Prognosis
Benign and malignant are
meaningless
with respect to brain tumors.
It is the
technical aspects that
determine the
prognosis
Haemorrhage and
midline shift
Raised ICP
~
As neoplasm grows contents of the

skull are compressed
~ Within the skull brain occupies 1400mls
CSF 100-200mls and blood 100-150mls
~ Displacement of CSF and blood compensate
initially for mass effect
~ Then ICP rises quickly mass effect
compression
vascular insufficiency
IC
ICP Herniations
~ Subfalcine herniation
~ Tentorial herniation
~ Tonsillar herniation
FALSE LOCALISING SIGNS

~ Occulomotor nerve compression
~ Abducens nerve compressed against
the petrous ligament
~ Ipsilateral hemiparesis from
compression of the cerebral peduncle against
the tentorium
~ PCA infarction from compression of the
artery against the tentorium
Under the age of 16, 75% occur

In the posterior fossa
~ Pilocytic astrocytoma
~ Ependymoma
~ Medulloblastoma
Medulloblastoma
Childhood
Male predominance
Medulloblastoma seeding down the cord
Ependymoma
~ childhood
~ Often occur in areas
where complete surgical
excision is
impossible
Neuroectodermal tumours
Prognosis depends on
a. Site
b. histology
Meningiomas
~ Older adults usually female
~ Increased incidence in Von Recklinhausen
disease
~ Association between meningiomas and breast
cancer
Meningioms
Clinical presentation depends on:
a) Site
b) Rapidity of growth
Prognosis ~ benign (usually)
~ slowly growing
~ often can be completely
excised
Meningioma
arising from the falx
cerebri
Spinal cord tumors

1. Extradural metastatic carcinoma,
myeloma, lymphoma
2. Intradural (extramedullary) meningioma
schwannoma
3. Intramedullary - gliomas
Metastatic tumours in Adults

~ Common
~ Over the age of 65 commonest
variety of intracerebral neoplasm
~ Mets in children uncommon but CNS
well recognised site for relapse of ALL
Case History
~ Patient transferred with a history of
Headaches and drowsiness
~ Microcytic hypochromic anaemia,
Thrombocytopenia
~ CT hydrocephalus no known cause
~ EVD inserted
~ IVH
~ RIP
E. O. N
Admitted on 02/10/00 with stridor and
Personality change
Progressive deterioration
Infective screen negative
? sCJD
??
Investigations
CSF NAD
MRI - ?
EEG - NAD
PM A50/01
Paraneoplastic encephalomyelitis
Tumour mass 8x6x4 cm, wt 120gms
Anterior, inferior and left lateral to the
Thyroid
Paraneoplastic encephalomyelitis
~ neurological disorders of unknown cause
associated with systemic malignancy
~ Subacute progressive course over mths years
~ May precede follow or occur simultaneously
with a systemic cancer
Include:
1. Encephalitis
2. Cerebellarr cortical degeneration
3. Myopathy
4. Peripheral neuropathy
5. Necrotising myelopathy
AB
Anti
Yo
Neurologi Cerebellar
deg
cal
synd
ICC
Anti HU
Anti Ri
Encephalo
myelitis
Opsoclonus- retinopathy
ataxia
Cytoplas PC Nuclei
and Ov ca
neurones
tumors
Anti
CAR
Nuclei of
Retinal
CNS
neurones,
neurones,bre rods, cones
ast and lung
ca
Benign cystic lesions in the brain

that may cause sudden death
~ colloid cyst of the third ventricle
~ Other cystic lesions
A209/01
CONCLUSION
1. Brain tumours classified into intrinsic,
extrinsic and spread from adjacent structures
2. Adults usually present with supratentorial tumours
3. Commonest primary tumour in adults gliomas. >65
metastatic tumours common
4. Paraneoplastic syndromes non-metastatic
complications of an underlying malignancy.

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TUMOURS OF THE CENTRAL

FM Brett MD., FRCPath

At the end of this lecture you

2. Extrinsic tumours arising from cranial and

The pathologist and CNS

~ Most primary tumors are sporadic and

Heritable syndromes with increased risk of CNS tumours

Type of CNS tumour

CNS neoplasms present with:

Sites of cerebral tumors

Factors in the aetiology of CNS neoplasms

1. Sex gliomas commoner in males

Prognostic factors in CNS tumors

Local destruction of neural tissue

Grading of Gliomas (WHO)

Predictive Markers in Malignant Gliomas

As neoplasm grows contents of the

FALSE LOCALISING SIGNS

Under the age of 16, 75% occur

Medulloblastoma seeding down the cord

Spinal cord tumors

Metastatic tumours in Adults

Benign cystic lesions in the brain

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