GLIAL CELLS.

NEURONS

GLIAL CELLS

Schwann Cell

Oligodendroc
yte

Astrocyte

Microglia

WHAT IS A BRAIN TUMOR?

PRIMARY VS. SECONDARY:

Primary

originates in the brain.

Children

Secondar
y

made up of cells that

have spread
(metastasized) to the
brain from somewhere
else in the body.
May lodge into the ff structures:
- Brain parenchyma most common area of
metastases
- Leptomeninges pia mater & arachnoid
- Dural space

Benign
slow-growing
Noncancerou
s
do not spread to
surrounding
tissue.

Maligna
nt
Cancerous
Fast-growing and
aggressive
can invade nearby
tissue and also are
more likely to recur
after treatment.

LOCALIZED VS. INVASIVE

Localized

Invasive

confined to
one area

spread to
surroundin
g areas

easier to
remove

more
difficult to
remove
completely

THEY CAN ALSO BE:

Extramedulla
ry
(Extraaxial)

Intramedullar
y (Intraaxial)

Meningioma

mostly
manifesting
as seizures

Pituitary
adenoma

Glioma

Vestibular
schwanom
ma

Primary
CNS
Lymphoma

Metastatic

Intraventricul
ar

WHO GRADING SYSTEM

Grade I-Pilocytic
astrocytoma

Benign cytological features-see

below

Grade II-Lowgrade astrocytoma

Moderate cellularity-no anaplasia

or mitotic activity

Grade IIIAnaplastic
astrocytoma

Cellularity, anaplasia, mitoses

Grade IVGlioblastoma

Same as Grade III plus

microvascular proliferation and
necrosis

WHO HISTOLOGIC CLASSIFICATION OF TUMORS OF THE

CNS
1.
2.
3.
4.

Tumors
Tumors
Tumors
Tumors
1.

5.
6.

9.
10.

Hemangioblastoma from primitive vascular

structures

Ex: Germinoma common in pineal gland area

Cysts and Tumor-like lesions

1.

8.

Neuroepithelial Tissue
Cranial and Spinal Nerves
the Meninges
Uncertain Histogenesis

Lymphomas and Hematopoietic Neoplasm

Germ Cell Tumor
1.

7.

of
of
of
of

Usually in the third ventricle

Tumors of the Sellar Regions

Local Extension from Regional Tumors
Metastatic Tumors

WHAT CAUSES A BRAIN TUMOR?

Being
male
Occupation
al
exposures

Family
history

Race

Age

OCCUPATIONAL EXPOSURES
Radiation

Formaldehy
de

Acrylonitrile

Vinyl
chloride

COMMON TYPES OF BRAIN

TUMORS

Astrocytomas come in four major subtypes:

juvenile pilocytic astrocytoma (grade 1)

fibrillary astrocytoma (grade 2)
anaplastic astrocytoma (grade 3)
glioblastoma multiforme (grade 4)

The higher the grade, the more aggressive

the tumor.

AGE INCIDENCE

Adults
-

Supratentorial: 80-85%
- Intratentorial: 15-20%

- Children
-

Intratentorial: 60%
- Supratentorial: 40%

CLINICAL PRESENTATION
Insidious
onset

Increased
ICP

Lateralizing or
focal neurologic
deficits

Headache

Slowly
Progressiv
e
Tumors
Mental,
behavioral and
personality

Seizure

CEREBRAL DYSFUNCTION

Seizure

Contralateral lesion:
Hemiparesis with Babinski reflex &
cranial nerve deficits
Hemisensory deficits
Homonymous
hemianopsia/quadrantanopsia

Language disorderaphasia
(motor area lesion: Brocas
aphasia; sensory lesion:
Wernickes aphasia)

Organic mental,
behavioral personality
changes

CEREBELLAR DYSFUNCTION
Hemisphere lesion

Vermis lesion

Ipsilatera
l limb
ataxia

Dysdiadochokine
sia

Intention
tremor

Truncal ataxia

Dysmetri
a

No limb ataxia

Brainstem
Dysfunction

INCREASED ICP

PAPILLEDEMA

COURSE OF ILLNESS

ANCILLARY PROCEDURES

Skull X-ray

EEG

Perimetry,
audiometry

Neuroimaging
studies

CSF examination

Biopsy

TREATMENT OF BRAIN TUMORS

Surgery

Brachytherapy

Radiotherapy

Biopsy

Chemotherapy

Gamma knife

THE NEUROLOGICAL
REHABILITATION TEAM:

The Rehabilitation Team

Neurologist

Neurosurgeon

Orthopaedist /
Orthopaedic
Surgeon

Physiatrist

Internist

Rehabilitation
Nurse

The Rehabilitation Team

Dietitian

Speech / Language
Therapist

Physical Therapist

Social Worker

Occupational
Therapist

Psychologist /
Psychiatrist ;
Recreational
therapist;
Case manager ;
Audiologist;

COMMON TYPES OF BRAIN

TUMORS

I. GLIOMAS
- Most common primary brain tumor
- 50% of all symptomatic brain tumors
- Incidence increases with advancing age
- Peak in 8th and 9th decades
- No known environmental factors
- No behavioral lifestyle choices
- Ionizing radiation: the only clear risk factor
- Originate from glial cells or their stem cell
precursors

GLIOMAS

Include:
a.

Astrocytoma
b. Oligodendroglioma
c. Ependymoma

- WHO Classification Basis

a.

Increased cellularity
b. Nuclear atypia
c. Endothelial proliferation
d. Necrosis

A. ASTROCYTOMA
- Most common glioma
- Cerebral astrocytoma (more in adults)

Behavioral changes
Seizures
Hemiparesis
Language difficulty

- Cerebellar astrocytoma (more in children)

-

Hemisphere
- Ataxia

- Brain stem (children)

-

Pons
- CN deficits

GRADE I:

Pilocytic Astrocytomas
Primary

in children &
young adults
Focal astrocytoma may
be associated with:
Neurofibromatosis type I
(NF-I)
Unusually excellent
prognosis

GRADE II:

Diffuse or Fibrillary
Astrocytoma
Most

common in the cerebral

hemisphere in young adults
Low grade or benign
histologically
Infiltrative usually a
problem because the tumor
cannot be resected
completely if this is a
characteristic of the tumor
Complete resection not
possible
Latent potential for
malignant transformation

GRADE III: ANAPLASTIC ASTROCYTOMA

GRADE IV: GLIOBLASTOMA MULTIFORME
Grades III and IV are high-grade gliomas
20% of all intracranial tumors
55% of gliomas
80% of gliomas of the cerebral
hemispheres in adults
Peak incidence middle to late adulthood
Males/females = 1.61
No familial predilection

ANAPLASTIC ASTROCYTOMA
Have increased pleomorphism, enlarged
nuclei and most importantly, increased
proliferative activity that is reflected as
increased mitotic activity.
There should be NO necrosis or endothelial
proliferation.
Presence of either/both is suggestive of
worse biological behavior.

GLIOBLASTOMA MULTIFORME

CSF seeding:
Malignant

cells in the CSF may form:

a. Distant foci in spinal roots

b. White spread meningeal gliomatosis

CSF

seeding implies that GBM can go to the CSF

spaces such as the subarachnoid space &
communicate with the ventricular system

Extraneural metastasis
-

To bone & lymph nodes (very rare) after a

craniotomy

Pseudopalisading around the necrosis is

common in GBM
Can cross the midline in a butterfly pattern:
this shows the aggressive nature of this

GLIOBLASTOMA MULTIFORME

IMAGING: HIGH- AND LOW-GRADE

GLIOMAS
High-grade or malignant gliomas: appear as
contrast enhancing mass lesions which arise
in white matter & are surrounded by edema
Low-grade gliomas: typically non-enhancing
lesions that diffusely infiltrate brain tissue &
may involve a large region of brain
Low-grade gliomas are usually best
appreciated on T2- weighted MRI scans.

PROGNOSIS OF ASTROCYTOMAS

Median survival
GBM:

1 year
Anaplastic astrocytoma: 3 years
Low-grade astrocytoma: 5 years
Others survive a decade or more
Most die from transformation of tumor to higher
grade

B. OLIGODENDROGLIOMA

Derived from oligodendrocytes or their

precursors
Oligodendrocytes

produce the white matter in

the brain

5-7% of all intracranial gliomas

Most often in the 3rd and 4th decades
Males:females = 2:1
Found primarily in cerebral hemispheres,
within the brain parenchyma
Highly infiltrative
May metastasize distantly in ventricular &
subarachnoid spaces like the GBM (CSF
seeding)

OLIGODENDROGLIOMA

FRIED EGG CELLS OF

OLIGODENDROGLIOMA

PROGNOSIS OF
OLIGODENDROGLIOMA

Median Survival
Low-grade

oligodendrogliomas: 8-16 years

Anaplastic oligodendrogliomas: 5 years
Tumors that have 1p/19q LOHbest prognosis
Many pxs die from malignant transformation of
the tumor

C. EPENDYMOMA
Arise from ependymal cells (an
intraventricular tumor)
More common in children

10%

pediatric intracranial tumors

5% of adult intracranial tumors

Most common in the 4th ventricle

Ataxia,

vertigo, increased ICP

May grow in brain parenchyma without

obvious attachment to the ventricular system
Spinal lesions more common in adults
Intracranial ependymomas predominate in
children

EPENDYMOMA

HISTOLOGICAL CHARACTERISTICS OF
EPENDYMOMA

Perivascular
pseudorosettes

Ependymal or
Homer-Wright
Rosettes

GFAP positive

Loss of
chromosome 22
particularly 22q

CSF seeding (drop

metastasis)

Cranial MRI

PROGNOSIS
5-year survival: 40-50%
10-year survival: 47-68%
Better prognosis:

Young

age
Infratentorial
Gross total excision
Low-grade histology

II. MENINGIOMA
Second most common primary brain tumor
Originate from arachnoid cells
(meningoepithelial cap cells normally seen in
arachnoid villi)
20% of all intracranial tumors (with
asymptomatic cases40% or more)
7% of all posterior fossa tumors
3-12% of cerebellopontine angle tumors

MENINGIOMA

II. MENINGIOMA
Most diagnosed in 6th % 7th decades
Female: Male3:2 to 2:1
Multiple in 5-15% (NF-2)
90% intracranial
10% intraspinal
Spinal meningioma: 10x in women
All familial meningiomas occur with NF-2
Rare in children (more in boys)

Rare with dural attachments

Usually Intraventricular or posterior fossa
Commonly with sarcomatous changes
Frequently with NF-2

ETIOLOGY OF MENINGIOMA

Radiotherapy

Head
trauma

Viral infection (SV-40)

Estrogen
receptors

PROGESTERONE RECEPTORS
-

Expressed in 80% of women with meningiomas

- Expressed in 40% of men with meningiomas

PATHOLOGY
Nodular tumors occasionally meningiomas en
plaque (sheer-like formation)
Highly vascular
Encapsulated and attached in the dura
(blood supply from external carotid artery)
Hyperostosis of adjacent bone (bone
proliferation)

HISTOLOGICAL CHARACTERISTICS
Benign
Typical features:

Whorls of arachnoid cells surrounding a central

hyaline material that eventually calcifies to form
PSAMMOMA BODIES
- No characteristic cytologic marker

CLINICAL MANIFESTATIONS
Some are asymptomaticfound incidentally by
MRI
But may have symptoms:

Tumor

location: by compression of underlying

neural structures
Sites of predilection
- Cerebral convexity (Sylvian & parasagittal areas)
- Falx cerebri
- Skull base
- Olfactory groove
- Sphenoid ridge
- CP angle
- Tuberculum sella

DIAGNOSIS

DIAGNOSIS

Cranial CT Scan
Isointense

or slightly hyperintense
Hyperostosis20%
Isointense (65%) or hypointense (35%) in T1 and
T2
Gadolinium

Angiography
Hypervascular

mass

embolization reduce the risk of intraoperative bleeding

MR Angiography & Venography

GROWTH RATE OF MENINGIOMA

Less than 1 cm per year (very slow growth
but can recur)
Tumor doubling time: 1.27 to 14.35 years

SURGERY
Complete excision may cure many
meningiomas
The extent of resection is the most important
in determining recurrence
For recurrence: reresection

RADIATION THERAPY
Residual tumor after surgery
Recurrent tumor
Atypical or malignant histology

III. TUMORS OF THE PITUITARY GLAND

Third most common primary
brain tumor
Often asymptomatic
Incidence at autopsy:
1.7 24%
Most common in adults in
the 3rd and 4th decade
10% incidence in children & adolescents
Not hereditary except MEN-1 (multiple
endocrine neoplasia)

PATHOLOGY

Microadenoma
-

Less than 1cm

- Symptoms due to excess hormone secretion (or
hyperfunctioning)
a. Growth hormone
b. Gonadotropin
c. Thyroid hormone
d. Adrenal hormone
e. Prolactin hormone

Macroadenoma
-

More than 1cm

- Symptoms due to compressing normal pituitary
gland and neural structure causing
hypofunctioning

PATHOLOGY

Endocrine Active (Secretory)

-

Prolactinoma

- Most common secretory intrasellar endocrine active

tumor
- Secreted either by microadenoma or macroadenoma

Growth hormone

- Before closure of epiphysis gigantism

- After closure of epiphysis acromegaly

ACTH: Cushings Syndrome

- FSH and LH

- Endocrine Inactive (Non-secretory or null

cell adenoma)
- 10% mixed secretory tumor

HISTOLOGICAL CHARACTERISTICS:
Almost all are histologically benign
Pituitary CA: rare
Macroadenomas
Pituitary Carcinoma

MACROADENOMAS
May invade dural bone
May infiltrate surrounding structure
Locally invasive pituitary adenomas are
nearly always histologically benign
Pleomorphism and mitotic figure insufficient
for diagnosis of carcinoma (may be seen in
benign adenomas)
Invasive character independent of growth
rate

PITUITARY CARCINOMA
Highly

invasive
Rapidly growing & anaplastic
Unequivocal diagnosis relies on
presence of distant metastasis

CLINICAL MANIFESTATIONS OF
TUMORS OF THE PITUITARY GLAND

Compression of neural and vascular structures

Headache
Hypopituitarism
Visual

symptoms

- visual loss
- visual field abnormality: bitemporal hemianopsia is the
most common

Papilledema

is rare
May enlarge with pregnancy
5% of pituitary adenoma present with pituitary
apoplexy

CLINICAL MANIFESTATIONS OF
TUMORS OF THE PITUITARY GLAND

Optic chiasm
-

Between hypothalamus & sella turcica

- When this is compressed bitemporal
hemianopsia

Optic nerve
-

When this is compressed ipsilateral blindness

Optic tract
When this is compressed contralateral
homonymous hemianopsia

Diaphragma sella
-

The dura that covers sella turcica

As tumor grows forward to the sella

compress the basal dura headache
affected pain-sensitive intracranial structures

VISUAL FIELD PATHWAYS

BITEMPORAL HEMIANOPSIA

IPSILATERAL BLINDNESS

CONTRALATERAL HOMONYMOUS
HEMIANOPSIA

HYPOTHALAMUS + THALAMUS
- Form the lateral wall of the 3rd ventricle
- Any pathology in the ventricular system will
cause accumulation of CSF proximal to the
block hydrocephalus

SUPRASELLAR REGION REGION OF

THE HYPOTHALAMUS

An example of a suprasellar tumor is a

craniopharyngoma in children & adults

A craniopharyngoma can compress the third ventricle

& cause the ff: (hydrocephalus with signs of increased
ICP)
- Headache
- Vomiting
- Papilledema
Nowadays, pituitary adenoma usually does not grow
until the region of the hypothalamus because visual
problems prompt consult & diagnosis.

Papilledema is also rare because it manifests late in

the course of the tumor. Before that happens, patient
must have been diagnosed already
Obstructive hydrocephalus: rare because of diagnosis
at visual problem level

PITUITARY APOPLEXY
- Hemorrhage or infarction of pituitary
adenoma
- Sudden onset of headache, nausea,
vomiting, visual loss, diplopia, altered mental
status
- Diagnosis by CT or MRI
- Treatment emergency surgery

DIAGNOSIS
- X-ray will show you ballooning of the sella
turcica
- Cranial MRI

Best way to evaluate pituitary pathology

TREATMENT

Surgery

Radiation
Treatment

Hormone
replacement

Radiosurgery

VIDEO ON ENDOSCOPIC
TRANSSPHENOIDAL SURGERY