TUMOURS OF CNS & PNS Seizures

Classification of Brain Tumour -

abnormal electrical
difficulty concentrating activity
-
lead to : loss i wnliousness

Primary secondary :
involuntary movement

( metastatic ) 10110 mental sharpness : loss i muscle control

Tumours Cell Of Origin Weakness / numbness Sign & Symptoms

Glioma Glial Cell
Germ tell Tumour German Headaches
Neuronal / Ganglion Tumour Neuronal / Ganglion tell diminished wnlioutn " '

Medullablastoma Poorly Differentiated Cell Nausea & vomiting

Lymphoma Lymphocytes
Ulli arachnoid
meningioma Meningothelial

Special characteristic of CNS tumour

locally invansive Spread of tumour Malignancy sensitive / critical area :
medulla oblongata
-

Spread via Re : midbrain

•

l at
ed :
subarachnoid space to cerebellum
-

CSF ( further areal

SG MALL Location
Grade o
te d t
I flow growing ,
non -

malignant , longterm Grading Re l a

survival adult :
supratentorial
11 slowgrowing recur as higher grade tumour
, .
Age
might be non malignant / malignant
-
children : intratentorial
111 malignant ,
returns higher grade tumour
IV
reproduce rapidly , aggressive malignant t .
Oligodendroglioma (G2) & Anaplastic Oligodendroglioma (G3)

Origin: Oligodendrocyte Age: 40 to 50 y/o Caused by IDH mutation of 1p and 19q Best prognosis: better than other
gliomas
Site: Cerebrum with predilection of white matter Treatment: Surgery
: Chemotherapy Survival rate: 15 to 20 years (G2)
: Radiation therapy : 10 to 15 years (G3)

Tumour in 4th ventricle

Compressing & infiltrating
surrounding structure

Ependydoma (G2) & Anaplatic Ependydoma (G3)

Origin: Ependymal cell Posterior fossa ependymomas often manifest with hydrocephalus
(obstruction of 4th ventricle)

Site: arise next to ependyma lined ventricular system

: In 2nd decades, near 4th ventricle
: In adults, arise at spinal cord (frequent in setting of neurofibromatosis type 2)
 Meningioma

Origin: Meningothelial cell from arachnoid Grading: I (Often, slow growing, prognosis excellent)
: Attached to dura mater : II (Rare, high recurrence, more aggressive)
: III (Rare, high recurrence, high mortality rate)

Gross morphology: Rubbery, rounded tumour Microscopically: Whorls/sheet like pattern neoplastic cell
: Compress underlying brain but easily seperated : Presence of psammoma bodies
: Solitary & encapsulated : Neoplastic cell have intranuclear inclusion

Clinically: No symptoms. Symptoms develop when tumors put pressure on specific part of brain
: S&S: seizures, changes in vision & hearing, weakness of arm
: Usually solitary but if present in multiple sites consider NF2
: Often express progesterone & estrogen receptor (female predominance)
: Risk factor: Radiation therapy of head & neck
Highly malignant Paediatric tumour
Prognosis: Very poor if untreated -appear primitive or undifferentiated Although
: Depends on amount of tumour resected neuronal and glial differentiation is encountered
Tumour radiosensitive -part of small round blue cell tumour (embryonal)
Survival Rate: Total excision & irradiation give 75% of
additional 5 years Age: children
S&S: Headaches
: Emesis (Vomiting) Sites: Cerebellum
: Lethargy
: Increased ICP
Complication: Dissemination through CSF (occlude CSF
flow causing hydrocephalus)
: May metastases to cauda equina

Medullablastoma
Microscopically: Extremely Cellular Gross Morphology: Well Circumscribed
: Small tumour cell : Gray
: Scanty cytoplasm : Friable
: Hyperchromatic nuclei
: Homer Wright rossetes with central neuropil
(blue arrow)
Neuronal/Ganglion Tumours

Classification
Ganglion Cell Tumors
Mixed Ganglion & Glial Cell
Tumours with neuronal
elements only
Other tumours with glial &
neuronal components
Examples
Gangliocytoma
Ganglioglioma
Cerebral neuroblastoma
Dysemembryoplastic
Neuroepithelial tumour

Primary CNS Lymphoma

Accounts for 2% of extranodal lymphomas & 1% of intracranial tumors

Most common CNS neoplasm in immunosuppressed individual (AIDS & Immunosuppression after transplantation)
Primary CNS tumors are diffuse B-cell lymphomas
- Aggressive
- Respond poorly to chemotherapy

Primary Germ tell Tumour

5 Types
:Sites: Midline o brain
: pineal gland & Suprasella
: Age: Young People (90%) Choriocarcinoma Embryonal carcinoma Dysgerminoma
: ~20y/o Yolk Sac Tumour Teratoma
Metastatic Tumor : 25% to 50% of intracranial tumor -Intraparenchymal
: Present clinically as mass lesion metastasis
: 5 common primary sites -Sharply demarcated
-Skin masses at junction of
-Kidney gray & white matter
-Lung (common origin of metastatic disease) surrounded by zone of
-Breast (main source of metastatic disease in women) oedema
-GIT
: Metastases to epidural or subdural space can cause spinal cord compression

Peripheral Nerve Sheath Tumor

*Arise from peripheral ner ve Schwann Cell * 3 important types Schwannoma

Perineurial Cell Neurofibroma
Fibroblast Malignant Peripheral Nerve Sheath Tumour

Cutaneous/Solitary Diffuse Neurofibroma Plexiform Neurofibroma

Neurofibroma
Occurence Sporadically & in NF1 NF1 associated Exclusively in NF1
patients patients
Risk of malignant Extremely small Rare Significant (5 to 10 %)
transformation
Site Cutaneous: Dermis & Large plaques like Large nerve trunks
subcutaneous fat elevations of skin

Solitary: Peripheral
Nerve
Gross Morphology Circumscribed skin lesion Diffuse but localized
involvement of skin & SC
Microscopically Composed of collagen &
spindled Schwann
 Malignant Peripheral Ner ve Sheath Tumors
- highly malignant tumors that are invasive
*multiple recurrence
*metastatic spread
- about 50% cases arise in setting of NF1
*from transformation of plexiform neurofibroma
*from radiation therapy
Inherited disease
-characterised by: Neurofibromas of peripheral
ner ves
: Gliomas of optic ner ve
: Pigmented nodules of Iris (Lisch
Nodules)
: Cutaneous hyperpigmented
macules (café au lait spots)
-Defect in Tumor Supressor Gene
Characterised by: Development of neoplasm &
hamartoma throughout body
: Linked with tumor suppressor gene
Familial Tumor Syndrome
Autosomal dominant
- Neurofibromatosis Type 1 (most common)
- Neurofibromatosis Type 2
- Tuberous Sclerosis
- Von Hippel Lindau Disease
-autosomal dominant syndrome
-less common
-resulting in range of tumor
*most commonly bilateral 8th nerve
schwannomas & multiple Meningioma