Klasifikasi Tumor Otak Div Neuroonko

KLASIFIKASI TUMOR
OTAK MENURUT WHO

2007 DAN 2016
Penyaji : Dr. Novy Rosalia Chandra

Divisi NeuroOnkologi RSMH
TUMOR OTAK
Didefinisikan :
Tumor intrakranial yang disebabkan pembelahan sel abnormal
dan tidak terkontrol ;
- Di Otak (neurons, glial cells (astrocytes,
oligodendrocytes, ependymal cells), lymphatic tissue,
blood vessels),
- Di Saraf Kranialis (myelin-producing Schwann cells),
- Di Meningen, Tulang Kepala (Skull), Kelenjar Pituitary dan
kelenjar pineal,
- Atau Tumor Metastase
KLASIFIKASI TUMOR OTAK WHO 2007
KLASIFIKASI TUMOR OTAK
(WHO, 2016)
GLIOMA
Berasal dari sel glia
Diklasifikasikan :
 Berdasarkan Tipe Sel
 Berdasarkan Grade
 Berdasarkan Lokasi
KLASIFIKASI
a) Berdasarkan Tipe Sel (Cell Type)

Glial Cells Glial Tumour
Astrocytes Astrocytomas
Oligodendrocytes Oligodendrogliomas
Ependymal cells Ependymomas
Different types of glia Mixed gliomas (oligoastrocytomas)
b) Berdasarkan Grade
Grade Differentiation Type Prognosis
Low-grade well-differentiated benign better

(not anaplastic)
High-grade undifferentiated malignant worse

(anaplastic)
C)Berdasarkan Lokasi
Type Location Site Age
Supratentorial above the tentorium in the cerebrum mostly in adults (70%)

Infratentorial below the tentorium in the cerebellum mostly in children (70%)
ASTROCYTOMA
Astrocytomas originate in star-shaped brain cells

called astrocytes
most common primary CNS malignancy
75% of neuroepithelial tumors
low grade  < 6 % of Intracranial tumors

grade (IV)  - 20 % of Intracranial tumors
- survival 3 - 12 months
Glioblastoma multiforme
standard name - “Glioblastoma”

- most common
- most aggressive
- 52% of all parenchymal brain tumor
- 20% of all intracranial tumors
Classified - Giant cell glioblastoma
- Gliosarcoma
Glioblastoma multiforme
Symptoms
depends highly on the location
asymptomatic condition
seizure, nausea and vomiting,
headache, and hemiparesis
progressive
memory, personality, or neurological deficit
due to temporal and frontal lobe involvement
Diagnosis
MRI ring-enhancing lesions
Definitive diagnosis of a suspected GBM on CT or MRI
stereotactic biopsy or
craniotomy with tumor resection
Oligodendroglioma
• a type of glioma that are believed to originate from the

oligodendrocytes of the brain or from a glial precursor cell.
• They occur primarily in adults (9.4% of all primary brain ),
in children (4% of all primary brain tumors)
• The average age at diagnosis is 35 years
• etiology of oligodendrogliomas is unknown

Oligodendroglioma
• First symptom - seizure

• Frontal lobe  affecting personality
• Headaches combined with increased intracranial pressure
• Depending on the location of the tumor  any
neurological deficit
(CT) or (MRI) scan is necessary to characterize the anatomy

of this tumor (size, location, heter/homogeneity)
Final diagnosis of this tumor  relies on histopathologic

examination (biopsy examination).
Neuroblastoma
most common extracranial solid cancer in childhood

most common cancer in infancy
50 % of neuroblastoma  children < 2 years old
neuroendocrine tumor,
arising from any neural crest element
of the sympathetic nervous system
most frequently originates in one of the adrenal glands

but can also develop in nerve tissues in the neck, chest,
abdomen, or pelvis
Neuroblastoma
One of the few human malignancies

known to demonstrate spontaneous regression from an
undifferentiated state to a completely benign cellular
appearance
three risk categories: - low

- intermediate
- high risk
Low-risk disease is most common in infants highly curable
with observation only or surgery
high-risk disease is difficult to cure, even with the most
intensive multi-modal therapies available
Medulloblastoma
highly malignant primary brain tumor

 originates in the cerebellum or posterior fossa
family of cranial primitive neuroectodermal tumors (PNET)
Infratentorial
All PNET tumors of the brain are invasive and rapidly
growing tumors
unlike most brain tumors spread through CSF
metastasize to different locations in brain and spine
 usually form in the fourth ventricle (between the
brainstem and the cerebellum)
 exact cell of origin  "medulloblast”
may be arised from cerebellar "stem cells" prevented from
dividing and differentiating of normal cell types
loss of genetic information on the distal part of
chromosome 17, distal to the p53 gene
Clinical manifestation
increased intracranial pressure blockage of the fourth

ventricle
repeated episodes of vomiting
morning headache
develop - stumbling gait
- frequent falls
- diplopia
- papilledema
- sixth cranial nerve palsy
- positional dizziness
- nystagmus
- facial sensory loss or motor weakness
Extraneural metastases to the rest of the body is rare

- only after craniotomy
Meningeal tumours
WHO classification
Tumour Grade
Meningioma I
Atypical meningioma II
Anaplastic / malignant III
meningioma
Haemangiopericytoma II
Anaplastic III
haemangiopericytoma
Haemangioblastoma I
Meningioma
second most common primary tumor of the CNS

arising from the arachnoid "cap" cells of the arachnoid villi in
the meninges
usually - benign
- can be malignant
Causes
some - familial
risk factor - radiation to the scalp
genetic mutations - inactivation mutations in the
neurofibromatosis 2 gene (merlin) on chromosome 22q
Signs and symptoms
Small tumors (e.g., < 2.0 cm)
- usually incidental findings at autopsy
- without having caused symptoms
Larger tumors
- symptoms depending on the size and location
Increased ICP eventually occurs

but is less frequent than in gliomas
Location being overlied Presentation
cerebrum Focal seizures

parasagittal frontoparietal region Progressive spastic weakness in legs and
incontinence
Sylvian tumors motor, sensory, aphasic, and seizure
symptoms depending on the location
Diagnosis
visualized with - contrast CT
- MRI with gadolinium
- arteriography
 lumbar puncture : protein is usually elevated
A contrast enhanced CT scan

of the brain
demonstrating
the appearance of a Meningioma
PITUITARY TUMOUR
10 – 50 % of all cranial tumour

Majority - benign
Metastasis - may occur
especially in elderly
One type of sellar region tumour

Differential diagnosis of sellar region mass
- craniopharyngioma
- meniongioma
- aneurysm
- Rathke’s cleft cyst
Clinical features
Mass effect - bitemporal hemianopia

- pressure on optic chiasma
- cranial nerve III, IV, VI disorder
- Endocrine disturbance
TUMOUR PRESENTATIONS
Prolactinoma Galactorrhoea
Amenorrhoea (P’,2’)
ACTH – producing tumour Cushing syndrome
GH – secreting tumour Acromegaly
gigantism
Tumours of the sellar region
Tumour Grade
Craniopharyngioma I
Granular cell tumour of the neurohypophysis I
Pituicytoma I
Spindle cell oncocytoma of the I
adenohypophysis
Metastatic brain tumor
A metastatic brain tumor is brain cancer that has spread from another part of the
body
Causes
Many tumor or cancer types can spread to the brain
most common lung cancer, 40 %
breast cancer, 10 – 30 %
melanoma, 5 – 15 %
kidney cancer,
bladder cancer,
certain sarcomas,
testicular and germ cell tumors,
and a number of others 15 %
Some types of cancers only spread to the brain infrequently - colon cancer,
very rarely - prostate cancer
INCIDENCE
more common than primary brain tumors
about one-fourth of all cancers that metastasize
10 - 30% of adult cancers
81 % - diagnosed after their primary cancer has been diagnosed and treated
35 % - have not been previously diagnosed
Lung, colon and renal cancers

- 80 % of metastatic brain tumors in men
Breast, lung, colon and melanoma cancers
- 80 % of metastatic brain tumors in women
incidence increased over time

- advances in neuroimaging procedures
- improvements in treatment of primary tumour and systemic disease
median survival - approximately one month without any treatment
two months with corticosteroids
three to six months with cranial irradiation
CHARACTERISTICS OF
METASTATIC BRAIN TUMORS
in the - cerebrum (80%),
- cerebellum (13-16%)
- brain stem (3%)
usually solid and spherical in shape

with well-defined margins,
center is often soft and filled with dead cells
zone of active tumor cells ( ringlike structure on the scan )
commonly grow in the junction of white and grey matter (most blood vessels)
50% of the time multiple tumors
usually accompanied by widespread edema
exact diagnosis - biopsy

sometimes recommended to eliminate the chance of misdiagnosis
Exams and Tests
examination - neurologic changes specific to the location of the tumor
- Signs of increased ICP
- Some -no symptoms until they are very large.
Then, rapid decline in neurologic function
Investigation
CT scan or MRI of the brain
confirm the diagnosis identify the location of the tumor. MRI - better
Cerebral angiography
show a space-occupying mass
which may or may not be highly vascular (filled with blood vessels).
chest x-ray, mammogram, CT scans of the chest, abdomen, and pelvis, and
other tests are performed to look for the original site of the tumor.
EEG may reveal abnormalities.
Histology - specimen from the tumor during surgery or
CT scan-guided biopsy is used to confirm the exact type of tumor
If the primary tumor can be located outside of the brain,
primary tumor is usually biopsied rather than the brain tumor.
lumbar puncture - to test the cerebral spinal fluid.
Treatment
depends on - size and type of the tumor

- initial site of the tumor
- general health of the person
goals of treatment - relief of symptoms
- improved functioning
- comfort
Radiation - whole brain is often used

- especially > one tumor
standard treatment - whole brain radiotherapy (WBRT)

Surgery - a single lesion and
when there is no cancer elsewhere in the body
- some completely removed
- deep or that infiltrate brain tissue
- debulked (reduce tumor size)
- reduce pressure and relieve symptoms in cases
when the tumor cannot be removed
Karnofsky Performance Scale
100 Normal; no complaints; no evidence of disease
90 Able to carry on normal activity; minor signs or symptoms of disease
80 Normal activity with effort; some signs or symptoms of disease
70 Cares for self; unable to carry on normal activity or to do active work
60 Requires occasional assistance but is able to care for most of needs
> 60 or 70, surgery could be considered, if other factors are favorable
Chemotherapy - not as helpful as surgery or radiation

for many types of cancer.
- does not pass the blood brain barrier
- metastatic from breast cancer : cyclophosphamide, 5-FU, and
methotrexate, Tamoxifen
Thank you..

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KLASIFIKASI TUMOR

OTAK MENURUT WHO

Penyaji : Dr. Novy Rosalia Chandra

a) Berdasarkan Tipe Sel (Cell Type)

Low-grade well-differentiated benign better

High-grade undifferentiated malignant worse

Supratentorial above the tentorium in the cerebrum mostly in adults (70%)

Astrocytomas originate in star-shaped brain cells

low grade  < 6 % of Intracranial tumors

standard name - “Glioblastoma”

• a type of glioma that are believed to originate from the

• etiology of oligodendrogliomas is unknown

• First symptom - seizure

(CT) or (MRI) scan is necessary to characterize the anatomy

Final diagnosis of this tumor  relies on histopathologic

most common extracranial solid cancer in childhood

most frequently originates in one of the adrenal glands

One of the few human malignancies

three risk categories: - low

highly malignant primary brain tumor

increased intracranial pressure blockage of the fourth

Extraneural metastases to the rest of the body is rare

second most common primary tumor of the CNS

Increased ICP eventually occurs

cerebrum Focal seizures

A contrast enhanced CT scan

10 – 50 % of all cranial tumour

One type of sellar region tumour

Mass effect - bitemporal hemianopia

Lung, colon and renal cancers

incidence increased over time

usually solid and spherical in shape

50% of the time multiple tumors

usually accompanied by widespread edema

exact diagnosis - biopsy

depends on - size and type of the tumor

Radiation - whole brain is often used

standard treatment - whole brain radiotherapy (WBRT)

Chemotherapy - not as helpful as surgery or radiation

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