NEOPLASMS OF THE

NERVOUS SYSTEM
DR NORAIDAH MASIR
DEPARTMENT OF PATHOLOGY
PPUKM
NEOPLASMS OF THE NERVOUS SYSTEM
Tumours are intracranial or intraspinal
Primary tumours or metastatic tumours

50-70% are primary tumours

CNS tumours accounts for 20% of all
childhood cancers
Primary nervous system tumours
1.Benign
2.Malignant
Based on histological features
Clinical features
NEOPLASMS OF THE NERVOUS SYSTEM
Majority are benign - low mitotic count, cellular uniformity,
slow growth.
But benign tumour may infiltrate large area of brain
leading to clinical deficits and poor prognosis.
Difficult to resect completely
May be located in a critical region eg in medulla causing
cardiorespiratory arrest.
Subarachnoid space provides a pathway for
spread into the CSF. Seedings along brain and
spinal cord
NEOPLASMS OF THE NERVOUS SYSTEM
LOCATION OF TUMOUR
Majority of childhood tumours arise in posterior
fossa.
Majority of adult tumours within cerebral
hemisphere above tentorium
Important to know the age of patient and
location of tumour for accurate histological
diagnosis
NEOPLASMS OF THE NERVOUS SYSTEM
EFFECTS AND COMPLICATION OF TUMOURS
Depends on location of tumor
Effect of space occupying lesion
Raised intracranial pressure
Headache, vomiting, loss of hearing, visual loss,
seizures
NEOPLASMS OF THE NERVOUS SYSTEM
EFFECTS AND COMPLICATION OF TUMOURS
1. Compression effect on adjacent neural tissues-
atrophy and loss of function.
2. Destruction of neural tissue due to tumour
infiltration.
3. Cerebral edema. Around tumour due to
neovascularisation with poorly developed BBB.
4. Irritative effects on neural tisues. Abnormal
stimulation of nerve epilepsy, seizures.
5. Hydrocephalus.
6. Increased intracranial pressure
NEOPLASMS OF THE NERVOUS SYSTEM
CLASIFICATION OF NERVOUS SYSTEM
NEOPLASMS
BASED ON WHO CLASSIFICATIONS
TUMOUR ARE GRADED ACCORDING TO
BIOLOGIC BEHAVIOUR
GRADE I
INDOLENT
GRADE II
GRADE III
AGGRESSIVE
GRADE IV
NEOPLASMS OF THE NERVOUS SYSTEM
CLASIFICATION OF NERVOUS SYSTEM NEOPLASMS
Based on WHO classifications
1. Gliomas - most common, arise from glial cells
2. Neuronal tumours - contain neuronal and glial elements
3. Poorly differentiated neoplasms - e.g. medulloblastoma
4. Other parenchymal tumours - lymphoma, germ cell
tumours
5. Meningioma - arachnoid meningiothelial cells
6. Metastatic tumours - mostly carcinomas
7. Peripheral nerve sheath tumours - schwannoma,
neurofibromas
NEOPLASMS OF THE NERVOUS SYSTEM
Gliomas
Astrocytomas Oligodendroglioma Ependymomas
Pilocytic astrocytoma - grade I
Well differentiated astrocytoma grade 1
Diffuse astrocytoma - grade II
Anaplastic astrocytoma - grade III
Glioblastoma - grade IV
NEOPLASMS OF THE NERVOUS SYSTEM
Astrocytomas
Well differentiated astrocytoma - grade I
Infiltrative slow growing.
Firm, white, ill-defined mass.
Histology: slight increase in cellularity, slightly
abnormal. Neurofibrillary proceeses.
Impossible to completely excise surgically because of
irregular extension.
Progress slowly.
Death 5-10 years after presentation.
NEOPLASMS OF THE NERVOUS SYSTEM
Astrocytomas
Pilocytic astrocytoma - grade I
Benign behaviour, slow growing
Children and young adults, 25% of intracranium
neoplasm in childhood
Cerebellum, wall of 3rd ventricle etc - location
Treatment resection. Rarely possible to completely
remove
NEOPLASMS OF THE NERVOUS SYSTEM
Astrocytomas
Pilocytic astrocytoma - grade I
Nodule of tumour in a cyst. Well circumscribed
NEOPLASMS OF THE NERVOUS SYSTEM
Astrocytomas
Pilocytic astrocytoma - grade I
Hypercellularity. Uniform fibrillary astrocytes.
Microcystic change. Bipolar cells with long, thin,
hairlike processes, fibrillary meshworks,
Rosenthal fibres (arrow).
NEOPLASMS OF THE NERVOUS SYSTEM
Astrocytomas
Infiltrative and distort
Diffuse astrocytoma - grade II
the brain
Bilateral expansion of septum
Right frontal tumour,
pellucidum by gray glassy
expanded, flattened gyri
tumour
NEOPLASMS OF THE NERVOUS SYSTEM
Astrocytomas
Infiltrative and distort
Diffuse astrocytoma - grade II
the brain
Mild to moderate increase in glial cellularity
Variable pleomorphism
GFAP-positive astrocytes
Symptoms depend on location and growth rate
Mean survival up to 5 years
NEOPLASMS OF THE NERVOUS SYSTEM
Astrocytomas
Anaplastic astrocytoma- grade III
Adults
More rapidly growing tumour.
An infiltrative white mass in cerebral hemisphere.
Microscopic: astrocytes, cellular tumour. More pleomorphic.
Neovascularisation. Increased mitotic rate. Higher proliferation rate
Mean length of survival : 15 months. Death after 1-5 years
Treatment : resection and radiation therapy
NEOPLASMS OF THE NERVOUS SYSTEM
Astrocytomas
Anaplastic astrocytoma- grade III
NEOPLASMS OF THE NERVOUS SYSTEM
Astrocytomas
Anaplastic astrocytoma- grade III
Microscopic: astrocytes, cellular
tumour. Mitosis. More pleomorphic
NEOPLASMS OF THE NERVOUS SYSTEM
Astrocytomas
Glioblastoma - grade IV
Highly aggressive
Poor prognosis
Mean length of survival : 15 months
Treatment : resection and radiation therapy
NEOPLASMS OF THE NERVOUS SYSTEM
Astrocytomas
Glioblastoma - grade IV
MRI - irregular large mass
in R parietal lobe
Necrotic, haemorrhagic
infiltrating mass
NEOPLASMS OF THE NERVOUS SYSTEM
Astrocytomas
Glioblastoma - grade IV
Cellular,
pleomorphic cells,
high mitosis, large
anaplastic cells,
necrosis, vascular
proliferation
NEOPLASMS OF THE NERVOUS SYSTEM
Gliomas
Oligodendroglioma
5-15% of gliomas
4th - 5th decade
Grade II
Several years of neurologic complaints
Seizures
Cerebral hemispheres - well
circumscribed gelatinous tumour
NEOPLASMS OF THE NERVOUS SYSTEM
Oligodendroglioma - grade II
Tumour cells with round nuclei and clear cytoplasm.
Thin walled capillaries
NEOPLASMS OF THE NERVOUS SYSTEM
Gliomas
Ependymomas
Ependyma-lined ventricular system and spinal cord.
Posterior fossa tumour - hydrocephalus dt
obsstruction of 4th ventricle
CSF dissemination occurs - poor prognosis
NEOPLASMS OF THE NERVOUS SYSTEM
Ependymomas
Tumour in the 4th Uniform cells with round
ventricle compressing oval nuclei. Form gland-
surrounding structure like structure
NEOPLASMS OF THE NERVOUS SYSTEM
Poorly differentiated neoplasms
MEDULLOBLASTOMA
In children
in cerebellum: most common in midline cerebellar vermis.
Derived form primitive neuroectodermal cells.
25% of childhood CNS tumours
Poorly / Undifferentiated tumour
Highly malignant
Poor survival if untreated
Treatment - resection and radiation - improves 5 year survival
NEOPLASMS OF THE NERVOUS SYSTEM
MEDULLOBLASTOMA
Grayish fleshy mass
with infiltrative margins
Medulloblastoma destroying superior cerebellum
NEOPLASMS OF THE NERVOUS SYSTEM
MEDULLOBLASTOMA
Highly cellular. Cellular sheets of small primitive
or undifferentiated cells. Hyperchromatic nuclei,
mitosis plenty
NEOPLASMS OF THE NERVOUS SYSTEM
MENINGIOMAS
Benign adult tumours - slow growing. Uncommon in children
Arise from meningiothelial cells of arachnoid
Attached to dura
Found on external surface of brain and within ventricular
system
Common site: parasaggital part of brain convexity, wing of
sphenoid, sella turcica, foramen magnum
Risk factor; previous radiation therapy
Symptoms- vague and nonlocalising or focal due to
compression of underlying brain
NEOPLASMS OF THE NERVOUS SYSTEM
MENINGIOMAS
Parasagital meningioma attached to dura,
compressing underlying brain
NEOPLASMS OF THE NERVOUS SYSTEM
MENINGIOMAS
Meningioma with
Psammoma bodies in
whorled pattern of
meningioma
cell growth
NEOPLASMS OF THE NERVOUS SYSTEM
SCHWANNOMA
Benign tumours from neural-cresst derived Schwann
cells.
Most intracranial tumours at cerebellopontine angle
attached to 8th cranial nerve (acoustic neuroma) -
tinnitus and hearing loss.
Sensory nerve are usually involved - painful lesion
NEOPLASMS OF THE NERVOUS SYSTEM
SCHWANNOMA
Bilateral 8th nerve Schwannoma
NEOPLASMS OF THE NERVOUS SYSTEM
SCHWANNOMA
Antoni B
Verocay bodies
Antoni A
Histology: mixture of 2 growth patterns. Elongated cells
in fascicles with high cellularity (antoni A) and loose
hypocellular areas (antoni B)
Other parenchymal tumours - lymphoma, germ cell tumours
Primary CNS lymphomas
1% of intracranial tumours
Immunossuppresion is a risk factor (eg. AIDS)
Most common type: diffusse large B cell lymphoma
Aggressive
Germ cell tumours of nervous sytem
Along midline
Pineal and supracellar region
< 1% of brain tumours
Teratoma - common congenital germ cell
tumour
Other types - germinoma, yolk sac tumour etc