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