TUMORS OF CENTRAL NERVOUS

SYSTEM

DR.SEWUNET M.TOLESSA
(ASST. PROFESSOR OF SURGERY WU )
 Outline
• Introduction CNS tumors
• Primary CNS tumors
- Incidence
- Etiology
- classification
- Clinical presentation
- Investigation
- Management
• Overview on
- metastatic tumor
- Spinal cord tumors
 INTRODUCTION
• Tumors of the CNS are tumors affect the brain and
spine
• Brain tumors encompass
– 1ry brain tumors -originate in the brain itself
– 2ndry brain tumor- Involve the brain as a metastatic site
• Primary tumors (benign or malignant)
• In adults ~ one-third (33 %)of tumors were malignant
• In children 65% were malignant
 INTRODUCTION

• Benign lesions often produce the same neurologic symptoms as

malignant tumors and distinguishing b/n the two based upon
clinical grounds can be difficult.
• Benign lesions a relatively small, slow-growing, mitotically inactive
tumor with little or no metastatic potential may lethal if located in
a region of the brain that renders it less than totally resectable
• Histological interpretation does not necessarily predict biological
behavior in the individual patient
• Most CNS tumors do not spread systemically ( but some
medulloblastoma )
• Both benign and malignant lesions are frequently treated similarly
with either surgical intervention or radiation therapy
• Younger patients have a significantly better prognosis compared to older
individuals
 Epidemiology
• 2ry tumors out number primary brain tumors by 10 to 1
and occur in 20% to 40% of cancer patients
• Spine cord tumors Represent 10-20 % of all primary CNS
tumours
• The annual incidence of CNS cancers is 18,500 (with 12,760
deaths)
• The incidence of primary brain tumors has increased
steadily with almost 25% increase during past two decades
• The incidence of primary brain tumors is higher in whites
than in blacks
• Morbidity & mortality is higher in males than in females
except for meningiomas and pituitary adenomas
 Epidemiology …….

• Has two peaks –at childhood & late adult hood

• Primary CNS tumors account for 10% of all neoplasm's in
adults
• In children Primary malignant CNS tumors are
– Account for 20 % of all childhood cancers
– 2nd most common childhood malignancies after hematologic
malignancies
– The most common pediatric solid organ tumor
– Leading cause of death from childhood cancer surpassing mortality
from hematologic malignancies
 ETIOLOGY
 not clearly understood
 cranial irradiation - (whole head irradiation for treatment of
tinea capitis, child hood leukemia's )
 carcinogenic chemicals - occupational studies electrical
workers (2% ) , agriculture workers(1.3 to 3.6-fold)
 infection and immunosupression - (viral infection, Simian
virus, Medulloblastoma, HIV/AIDS, toxoplasma infection)
 head trauma - (meningioma)
 genetic factors - inaactivation or over expression gene
controlling growth factors, e.g. loss of p53
 inherited factors -about 5% had a family history of brain tumo
and associated with several familial syndromes
Familial syndrome associated with CNS tumors
SYNDROME ERRORS CNS TUMOR
VON HIPPEL-LINDALI GERM LINE MUTATION OF HEMANGIOBLASTOMA
THE VHL GENE
LI-FRAUMENI GERM LINE MUTATION IN ASTROCYTOMA, PNET
ONE P53 ALLELE (1RY NEUROEPITHELIAL
TUMOURS )
TURCOT SYNDROME ADENOMATOUS GLIOBLASTOMA &
(BOURNEVILLE'S POLYPOSIS GENE MEDULLOBLASTOMA
TUBEROUS SCLEROSIS,
BTP SYNDROME)
VON RECKLINGHAUSEN'S ACTIVATING GTP OPTIC GLIOMA,
NEUROFIBROMATOSIS HYDROLYSIS ON RAS ASTROCYTOMA ,NEUROFI
TYPE I PROTEINS BROMA
NEUROFIBROMATOSIS ACOUSTIC NEUROMA,
TYPE II MENINGIOMA,EPENDYMO
MA, ASTROCYTOMA
 CLASSIFICATION CNS TUMOR

• Histogenesis
– Glial Vs Non-glial
• Histologic grading
– Benign Vs Malignant
• Anatomic location
– Intra axial Vs Extra axial
– Supratentorial Vs infratentorial
– Pituitary, Pineal region, Posterior fossa,…
CLASSIFICATION ……….
1.Based on cell of origin
A. primary tumors of the brain are divided into
1. Intra-axial
 Arising from neuroepithelial cells of brain parenchyma (Glial Tumors, Neural
Tumors and Mixed Tumors ) –50%
 Astrocytomas , most common glial tumors (90%)
 Oligodendroglioma accounts about 10% of gliomas
 Primitive neuroectodermal tumors arise from bipotential cells,
capable of differentiating into neurons or glial cells, most
common medulloblastomas
2.extra-axial-
 Arising from outside the brain parenchyma, meninges or crianal and spinal
nerves
 Include meningiomas (15-18%), pituitary
adenoma(8%) ,neurinomas /schwannomas (6%)
B. Metastatic - ( mainly from lung, breast, GI, kidney, melanoma) 15%
CLASSIFICATION ……….
CLASSIFICATION ……….
 CLASSIFICATION ……….

• WHO classification: identifies 7 groups.

– Tumors of Neuroepithelial tissue
– Tumors of cranial and paraspinal nerves
– Tumors of the meninges
– Lymphomas and hematopoietic neoplasms
– Germ cell tumors
– Tumors of the sellar region
– Metastatic tumors
2
The incidence of common intracranial tumors in adults
Astrocytoma 45-50%
Low-grade (benign) astrocytoma: 25-30%
High-grade (malig.) Astrocytoma: 50-60%
Oligodendroglioma: 5%
Ependymoma: 5%
Medulloblastoma: 6%
Meningioma: 15%
Schwannoma: 6%
Pituitary adenoma: 5%
Craniopharyngioma: 3%
Metastatic tumors: 20-30% of all intracranial
tumors
 Frequency of Primary CNS Tumors
Children (0–14 Years) Percentage Adults (≥15 Years) Percentage
Glial
 Glioblastoma 20 Glioblastoma 50
 Astrocytoma 21 Astrocytoma 10
Ependymoma 7 Ependymoma 2
Oligodendroglioma 1 Oligodendroglioma 3
Medulloblastoma 24 Medulloblastoma 2
Neuroblastoma 3 Neurilemmoma 2
Neurilemmoma 1 Pituitary adenoma 4
Craniopharyngioma 5 Craniopharyngioma 1
Meningioma 5 Meningioma 17
Teratoma 2 Pinealoma 1
Pinealoma 2 Hemangioma 2
Hemangioma 3 Sarcoma 1
Sarcoma 1
Others 5 Others 5
 Tumour Types: Age, and Location
Supratentorial Infratentorial
Children (< 15 astrocytoma - all grades e.g. cerebellar astrocytoma
yrs, primarily optic nerve astrocytoma medulloblastoma
infratentorial - craniopharyngioma ependymoma choroid plexus papilloma
80%) other:dermoid/epidermoid, pineal brain stem astrocytoma
tumours, primitive
neuroectodermal tumors
Adult (> 15 astrocytoma (40-50%) Most -metastatic (20-
years, primarily metastatic (20-30%) meningioma 30%) schwannoma (6%)
supratentorial - (15%) pituitary ademona (5%) e.g. acoustic neuroma
80%) oligodendroglioma (5%) Hemangioblastoma
medulloblastoma (5%)
ependymomas
CLINICAL MANIFESTATIONS OF
INTRACRANIAL
NEOPLASMS
caused by
CLINICAL MANIFESTATIONS OF INTRACRANIAL NEOPLASMS

A. SYMPTOMS DUE TO INCREASED ICP

Headache
Vomiting
Papulloedema
False localizing signs
 Depression of conscious level
Signs of tentorial herniation and coning
Epilepsy
B .SYMPTOMS DUE TO LOCAL DYSFUNCTION
Depends on tumor locations;
(1) Hemispheric
(2) specific lobes
(3) sites within specific lobes,
(4) multiple lobes,
(5) lobar vs nonlobar,
(6) brainstem or thalamus or basal ganglia vs hemisphere,
(7) deep vs superficial
(8) supratentorial vs infratentorial.
 Supratentorial tumors commonly present with focal neurologic deficit
 Infratentorial tumors often cause increased ICP due to hydrocephalus from
compression of the fourth ventricle
 Local effects in posterior fossa
 Cerebellar hemisphere or brain stem dysfunction) extremity ataxias, truncal
ataxia, cn palsy - often multiple, nystagmus, loc. Long tract signs
 Rarely cause seizures
Median section
 Diagnosis of brain tumors
• The proper evaluation of the patient with a suspected
brain tumor requires
- a detailed history,
- comprehensive neurologic examination, and
- appropriate diagnostic neuroimaging studies
• An understanding of neuroanatomy is the foundation of
comprehensive neurologic examination and diagnosis
• Help - Where is the lesion?
- What is the lesion?
- Determine the etiology of the problem
 NEUROIMAGING
Is the major diagnostic modality in the evaluation of brain tumors
and differciate it from non-neoplastic conditions presenting with
similar signs and symptoms

Plain x-rays
Skull X-rays are rarely used in neurosurgery, with advent of CT
Scan
• the presence of calcified brain lesions( oligoderoglioma)
• hyperostosis of adjacent bone in mengioma, or tumors involving
the skull, and
• abnormalities in the size and shape of sella turcica, which suggest
large pituitary tumors.
• chronically Increased ICP , accompanied by thinning of the
dorsum sellae
Computed tomography
CT has largely been replaced by cranial MRI as the imaging
modality of choice for brain tumors
However, CT retains utility in selected situations:
 To detect metastases to the skull base or vascular
involvement
 the first investigation for cerebral trauma and
suspected intracranial bleeding and detection and
localization intracranial mass lesion To unstable patient
(to screen )
Superior in demonstrating calcification and bone
abnormalities
Those unable to undergo MRI (implanted pacemaker,
metal fragment, paramagnetic surgical clips) or
Unwilling (b/c claustrophobia)
 Magnetic resonance imaging (MRI)

• Is the gold standard modality for diagnosis, presurgical

planning, and post-therapeutic monitoring of brain tumors
• Advantages
 superior to CT in permitting visualization of the tumor and its relationship
to the surrounding normal parenchyma , and for defining the vascular
distribution of the abnormality
 provide information that indicates the specific tumor type
 Contrast use shows BBB disruption and tumor aggressiveness
 No radiation ,no contrast exposure
 Show lesions of post fossa clearly

• Disadvantage
– poor for demonstrating fractures in dense cortical bone, and intra-
lessional calcification emit no signal on MRI image
– very sick
T2 WI are most sensitive for tumor and edema extent
 Positron emission tomography (PET Scan)

• Principle
– Tests metabolism by indicating
uptake of radio labeled glucose.
– During specific actions, certain parts
of the brain show increased activity
on PET

• Utility of PET Tumors scans

to detect malignant tumors with high
metabolic rates.

This information can guide the neurosurgeon to biopsy tumor,

mapping of functional areas of the brain prior to surgery or
radiation locations with the most aggressive biologic behavior
 Cytological evaluation
 Histological diagnosis important for confirmation of (CNS)
tumor
 specimen obtained
 open surgical procedure- is the preferred to obtain adequte
tissue for a histologic diagnosis
 stereotactic biopsy techniques - guided by (MRI) or (CT),
preferred tumors deeply located in the brain are not amenable
to open surgical intervention because of the high risk of
irreversible neurological impairment
 cerebrospinal fluid
 Cerebrospinal fluid (CSF) Analysis
• Preformed only after ruled out increased ICP or papilledema
or in patients with suspected intracranial mass lesions,
• can provide useful diagnostic information To obtain cells for
cytologic examination when
- carcinomatous meningitis (seeding of the meninges with
neoplastic cells) is a diagnostic possibility.
- leukemic involvement of the CSF is the most common
malignant diagnosis, if cells consistent with immunoblasts are
present
• Tumor markers: carcino-embryonic antigen/CEA, alpha-
fetoprotein ,pineal region germ cell tumors
 Management

• a multimodal approach of - surgery,

- radiotherapy, and
- chemotherapy
- pharmacological Rx
• preoperative and perioperative complications
have to be addressed medically first:
- Increased intracranial pressure (ICP)
- Seizures
 Management
• Raised ICP
- steroids useful for vasogenic cerebral edema
- elevate head of bed at 30-45 degrees ––> decreases intracranial
venous pressure
- ventilate/hyperventilate decreases pCO2, increases pO2, decreases
venous pressure
• mannitol can be given Urgent situations , effects in 30 minutes,
• Anti-convulsants FOR patient has seized , prophylactic undergoing
craniotomy
• pharmacological treatment
for pituitary tumors
– (functioning Prolactin Tumors, Dopaminergic drugs (Bromocriptine)
to Restore fertility , Impotence ,Decreased libido , Preserve
pituitary function, Reduce tumor bulk, Prevent osteoporosis)
 Surgical management
• The major objective of surgery is
– to resect and potentially cure the tumor; But For many
tumors surgical cure is not possible
– Maximal tumor reduction with minimal risk to damage
neurological function
– Internal decompression - rapidly reduce tumor bulk with
potential benefits in terms of mass effect, edema, and
hydrocephalus and
– provide tissue for histological diagnosis
– remove CSF by external ventricular catheter drain or
shunting prior to craniotomy , reducing the potential risk
of herniation , Postoperatively, allows drainage
 Surgical management
• Approach
 Craniotomy-
 Transphenoidal route –through sphenoid sinus to acess the pituitary fossa
/lesion of the sella
 Transoral route-followed by removal of arch of the atlas odonatid peg &
clivus access to anterior aspect of the brain stem and upper cervical cord
 Craniectomy- burr hole followed by removal surrounding bone to increase
exposure, used approach posterior fossa
 stereotactic techniques - with microneurosurgery aprouch
– conventional stereotaxic frame
_frameless stereotoxy to take biopsy , brachytherapy , resection
in accessible tumors , amygdalohippocampectomy in rx epilipsy,
aspiration of abcess
A frontotemporal craniotomy

-A standard curvilinear
frontotemporal skin incision is
made behind the hairline
-The frontal bur hole on eternal
orbital process

The head of the bed is raised 20°

and the patient’s head is rotated
30° away from the side of surgery
 Surgical
management
Transsphenoidal
Surgery

• complete • partial
• Meningiomas • Gliomas in the
• pituitary tumours frontal, occipital and
• acoustic neuromas temporal poles
• some solitary
metastases
 Radiation Therapy
• An integral role in the treatment of most malignant and
many benign primary CNS tumors.
– As definitive treatment in more radiosensitive diseases
such as embryonal tumors, PNET and germ cell tumors,
– As adjuvant treatment to halt further tumor growth in
schwannomas, meningiomas, pituitary tumors, and
craniopharyngiomas.
• Decrease local failure
• Delay recurrence
– Radiation therapy is also the primary modality in
palliating brain metastases, Prolong survival in gliomas
 Radiation Therapy
• Methods
 conventional /conformal radiotherapy- repeated
administration of small doses of radiation to a relatively large
target, as in whole brain or focal RT
 Stereotactic radiotherapy- multiple converging beams from a
linear accelerator focused on selected target
 Interstitial technique / brachytherapy– treated by
implantation of multiple radio-active seeds
 Proton therapy – deliver of high dose of radiation to very
localized region
 Chemotherapy
• As a primary treatment is questionable because of toxicity
and drug access BBB
• But reserve to
– Tumor recurrence after radiation or surgery
– useful as primary treatment for lymphoma and medulloblastoma
– as adjunctive therapy for oligodendroglioma and some high-grade
astrocytomas
• Effectiveness increased
– Intra-arterial injection of glioblastomas
– BBB may be iatrogenically disrupted by giving mannitol before
chemotherapeutic delivery has been shown to affect malignant
tumor response.
– Intrathecal adminstration of agent via LP ventricular acess
summary
 Metastatic Tumors

• The most common brain tumor (>half )

• The disorders usually appear in patients with advanced metastatic disease,
although they may be the first symptom of cancer( 10 %)
• Common sources
– Lung 50 % Breast 33%
Colonic 9% Melanoma 7%
• mostly reach the nervous system through the hematogenous route
• They may represent local extension of regional tumors like chordoma or scalp
cancer
• Usually seed the gray-white junction, Mostly frontal and parietal lobes
• Other common locations are the cerebellum (it is the most common p-fossa
tumor in adults)and the meninges (carcinomatous meningitis, also known as
leptomeningeal carcinomatosis )
 Metastatic Tumors
• The presence of brain metastases should be suspected in all
cancer patients who develop neurological symptoms
• MRI is the study of choice for evaluation
• These lesions are typically well circumscribed, round, and
multiple.
• Among solitary lesion on CT/MRI 7% metastatic, 87% primary CNS
tumor and 6% non-neoplastic
• Such findings should prompt a metastatic work-up, including CT
scan of the chest, abdomen, and pelvis, and a bone scan.
 Metastatic Tumors management
 With optimal rx , median survival is only 26-32 wks so management mostly
palliative
 Management largely depends upon - the primary tumor, overall tumor
burden, patient's medical condition/performance states and location and
number of metastases.
 THE KARNOFSKY SCALE
100 Normal—no evidence of disease
90 Minor symptoms—able to carry on normal activity
80 Some symptoms—normal activity with effort
70 Unable to carry on normal activity—cares for self
60 Cares for most needs—requires occasional assistance
50 Requires considerable assistance
40 Disabled
30 Severely disabled
20 Active supportive treatment needed—very sick
10 Moribund
SPINAL CORD TUMORS
- Lesions that occur with in or adjacent to the spinal
cord.
- Represent 10-20 % of all primary CNS tumours
- can be primary( most are benign) or metastasis

CLASSIFICATION
Intra spinal tumours
relationship to the Dura a. Extradural
b. Intradural
Intradural by relationship to the spinal cord
b-1. Intramedullary
b-2. Extramedullary
EXTRADURAL TUMOURS
 Extradural tumors are largely metastatic
 Account for approximately 55% of spinal tumors .
 Arise from verteberal bodies or epidermal tissue or metastatic
(most commonly) particularly from
– Prostate- 90% - Breast – 74 %
- Lung – 45%
 Metastasis to - Thoracic – 50 %
- Lumbosacral – 30 %
- Cervical – 20 %
 Primary Tumors –rare Hemangiomas, Osteoblastic lesions include osteoid
osteoma and osteoblastoma, Ewing's sarcoma, osteosarcoma,
chondrosarcoma, and plasmacytoma
INTRADURAL
 45 % of of spinal tumors
 Most are primary SC
 Approximately 29 percent of intradural tumors are
intramedullary, and approximately 71 percent are
extramedullary
EXTRAMEDULLARY
 These tumors are located within the dura, but outside the substance of the spinal cord
 Typically seen as well circumscribed lesions , rarely invade the
neural axis
 Benign in over 90 %
 Approximately 70 percent are either neurilemmomas or
meningiomas
 Includes
o Nerve sheath tumours (schwannomas and neurofibromas)
o Meningiomas
o Metastatic tumors may also seed this area through subarachnoid
spreading (lymphoma, ependymoma, medulloblastoma)
INTRAMEDULLARY TUMOURS
– 29 % of intradural tumors
– Originate from the substance of the spinal cord
– Approximately 59 percent are either ependymomas (28
percent) or astrocytomas (31 percent)
– Majority
• Ependymoma
• Astrocytoma
• Metastasis from primary brain tumor via CSF , drop
metastasis. e.g. PNET and MEDULLOBLASTOMA, ependyoma,
germ cell tumours...
 Intradural spinal cord tumors

Intradural tumors Percent

Intramedullary
Ependymomas 8
Astrocytomas 9
Others (each less than 2 12
Total 29

Extramedullary
Neurilemmoma 27
Meningioma 23
Sarcoma 10
Others 11
Total 71
The effects of spinal tumors
 Destruction of bones or ligaments can cause spinal
instability, leading to deformities such as kyphosis,
subluxation, or possible subsequent neural compression
 Tumor growth can cause direct compression of the spinal
cord , causing myelopathy, or the nerve roots, causing
radiculopathy
Factors that determine the clinical picture include
 The position of the tumor with respect to the transverse
plane of the spinal cord (dorsal, ventral, lateral, or
central);
 The position of the tumor with respect to the
longitudinal plane (cervical, thoracic, lumbar, or sacral)
 Tumor effects on the blood supply of the spinal cord
 the histologic nature of the lesion
INVESTIGATION
- Plain X- ray - collapse
- loss of pedicle
- sclerosis
- widening of foramina
- soft tissue shadow
- Myelography - not extent of invasion
- relation to cord or dura
- CT – scan – for doing biopsy in extradural tumours
- MRI – investigation of choice
- provide excellent delineating of SC & surrounding structure
- enhance almost all intrinsic SC tumours & metastases
- same may spread through out the CSF –Whole neuraxis
imaging.
 MANAGEMENT

 OPERATIVE MANAGEMENT
– Progressive neurologic loss constitutes a surgical emergency
– Surgical removal Followed by Postoperative Radiation Therapy
– Surgery is likely to improve quality of life for patients with life
expectancy of 6 months or more
– The surgical approach is directed toward the tumor area
• Laminectomy posteriorly tumors
• vertebrectomy a posterolateral thoracotomy
• Thoracoabdominal approach
– For high grade lesion pos-op RTX(+/-chemotherapy)
 REFERENCES
 Hand book of Neurosurgery-seventh
edition
 Text book of clinical neurology third
edition
 European Society for Medical Oncology
Handbook of Advanced Cancer Care
 Shwartz principles of surgery-9th ed.
 Sabiston text book of surgery -16th ed.
 UPTodate