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
Li- 17q13 p53 glioma
Fraumani
Gorlin’s 9q31 PNET
syndrome
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. Local destruction of neural tissue

2. Oedema
3. Distortion of neural tissue
4. 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 Anti HU Anti Ri Anti
Yo CAR

Neurologi Cerebellar Encephalo Opsoclonus- retinopathy

cal deg myelitis ataxia

synd
ICC Cytoplas PC Nuclei Nuclei of Retinal
and Ov ca neurones CNS neurones,
tumors 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.