Brain tumor

Dr Ibrahim Abdel-Aal
lecturer of neurosurgery
INTRACRANIAL TUMORS (ICTS)

Primary or Metastatic
Occur with equal frequency in adults, but in
children primary tumors are far more common.
Primary ICTs account for
~2% of cancers in adults
20% of all cancers in children.
INTRACRANIAL TUMORS (ICTS)

Because of its location, a benign ICT may have
fatal malignant effects.
Malignant ICTs spread by:
Direct infiltration of adjacent tissues
May disseminate within the CNS via CSF.
Gliomas account for 60% of primary ICTs
Meningiomas for 20% & all others 20%.
All CNS tumors behave as malignant clinically .
The unique features of CNS tumors ICP

1. CNS tumors- < 2% of all malignant tumors.
They grow in a unique environment: the
intracranial space.
2. The intracranial contents - incompressible Brain
and blood contained within a rigid unyielding bony
structure.
3. Intracranial pathologies (tumors, abscess,
hematoma, infarction, edema, etc.) eventually
produce life threatening increase of the
intracranial-pressure: ICP.




Tumor location
Intraaxial versus extraaxial
Supratentorial versus infratentorial
Locations vault versus skull base
intraventricular
cistern
brain stem
cerebellar
orbital
WHO Histologic Classification of CNS
tumors:
Neuroepithelial Tumors
Astrocytic tumors
Oligodemdroglial tumors
Ependymal tumors
Mixed gliomas
Choroid plexus tumors
Neurologic tumors
Pineal parenchymal
tumors
Embryonal tumors
Tumors of cranial/spinal
nerves
Mesenchymal tumors,
benign
Mesenchymal tumors,
malignant
Uncertain histogenesis
Hemopoietic neoplasms
Cysts/tumorlike lesions
Sellar tumors
Neuroectodermal tumors
Glial cells:
astrocytes (A) - Astrocytoma
Oligodendroglial cells - Oligodendroglioma
Ependymal cells Ependymoma

Neurons Gangliocytoma
ASTROCYTOMAS
most common primary ICTS in adults
MC in the cerebral hemispheres
MC Symptoms: headaches, seizures, focal
neurologic deficits
Seen in kids with increased cellularity
Low-grade Astrocytomas:

Gross:
Poorly defined gray-white infiltrative tumors.
Histology:
Hypercellularity; astrocytic nuclei of mild degree of
atypia & astrocytic processes fibrillary
background = fingers of astrocytes

Low-grade Astrocytomas
Pilocytic Astrocytomas:
MC in the cerebellum of children & young adults; and
less commonly in the optic nerve, hypothalamic
region or cerebral hemispheres
Morphology:
Cystic, with a tumor nodule in the wall of the cyst.
Composed of bipolar astrocytes, with long hair-like
processes, Rosenthal fibers & Micro-cysts+
calcification = good prognosis
Grow very slowly (some patients have survived for
>40 yrs after incomplete resection) & have an
Excellent prognosis
DD ;- not to confuse with low grade Fibrillary
Astrocytoma
Juvenile pilocytic astrocytoma
Pilocytic Astrocytoma
Gr. III. astrocytoma
Gr. IV. astro ~ = GBM
Gr. IV. astro ~ = GBM
Gr. IV. astro ~ = GBM
GBM: necrosis/pseudo-palisade
GBM: pleomorphic cytology
OLIGODENDROGLIOMA
Comprise ~ 4-8% of Gliomas
Arise in the cerebral white matter
MC in the 3th & 4th decades
Gross:
Well circumscribed, gelatinous, gray masses,
with foci of hemorrhage & calcification.
Grows slowly, presents commonly with
seizures, prognosis is better than
Astrocytoma, average survival is 5-10 yrs
(with modern therapeutic approaches
EPENDYMOMA
Arise from the Ependymal lining of the ventricles or
the central canal of the spinal cord
Arise in the
Fourth ventricle in children & young adults
Spinal cord in the middle aged.
Morphology:
Highly cellular, tumor cells have regular nuclei
May exhibit epithelial features with formation of rosettes
(Flexner) or canals, also perivascular pseudo-
rosettes (homer )
Most tumors are well differentiated
4th ventricle tumors:
May cause hydrocephalus, usually cant be completely
removed
CSF dissemination may occur
Average survival is ~ 4 yrs
Myxo-papillary Ependymomas
Arise in the filum terminale of the spinal cord
Prognosis depends on completeness of
surgical excision

Ependymoma
Rosettes & perivascular Pseudo-rosettes
MEDULLOBLASTOMAS
Second MC ICT of childhood (after Astrocytomas).
Occurs exclusively in the cerebellum.
Derived from fetal external granular layer of
cerebellum.
Grows rapidly & occludes CSF flow hydrocephalus.
Seeds through CSF implants around the spinal
cord & cauda equina (need irradiation of the whole
Neuraxis).
Histology:
Extremely cellular, anaplastic, small round or carrot-shaped
cells with hyperchromatic nuclei, N/C, may form Homer-
Wright pseudo-rosettes
Highly malignant, yet radiosensitive & 5-yr survival
75%.
Medulloblastoma
Medulloblastoma
Homer-Wright pseudo-rosettes
carrot-shaped cells
MENINGIOMAS
Usually Benign slow-growing tumors of adults, F/M 3:2
Originate from meningothelial cells of the arachnoid.
Usually solitary ( multiple meningiomas NF2 )
Morphology:
Firm rounded masses, adherent to the dura and
compressing the underlying brain (no infiltration).
Histologic variants include:
Syncytial, fibroblastic, transitional, Psammomatous &
papillary ( propensity to recur).
Malignant Meningioma is very rare
Infiltrates the underlying brain, shows marked nuclear atypia,
mitoses, & foci of necrosis.
Other rare sarcomas of meninges include:
Hemangiopericytoma, malignant fibrous histiocytoma &
Fibrosarcoma.
Meningioma
Meningioma
Pituitary tumors

Epidemiology

10-15% of all primary brain tumors
20-25% of pituitary glands at autopsy
found to have adenomas
70% of adenomas are endocrinogically
secreting
25% of those with MEN-I develop pituitary
adenomas
Natural History
Pituitary adenomas have long natural
history
Vary in size and direction of spread
Microadenomas < 10 mm may cause
focal bulging
Macroadenomas > 10 mm cause
problems due to mass effect


Clinical Presentation
Most common are endocrine abnormalities
hyper-/hyposecretion of ant. pituitary
hormones
HA
Vision changes bitemporal hemianopsia
and superior
temporal defects


Endocrine-Active Pituitary
Adenomas
Prolactin Amenorrhea, galactorrhea,
impotence
Growth hormone Gigantism and
acromegaly
Corticotropin Cushings disease,
TSH - Hyperthyroidism
Pathologic Classification
Benign or malignant
Chromophobic Non-functioning
Basophilic Cushings
Acidophilic - Acromegaly
Mixed
WHO Classification
Five-tiered system
Clinical presentation and secretory activity
Size and invasiveness (e.g. Hardy)
Histology (typical vs. atypical)
Immunohistologic profile
Ultrasturctural subtype
NERVE SHEATH TUMORS
1. Schwannomas:
Benign tumors of Schwann cells
MC in the vestibular branch of the VIII CN at the cerebello-
pontine angle (acoustic neuroma) tinnitus & hearing loss
Also involve branches of the trigeminal nerve & dorsal nerve
roots
Tumors are encapsulated, attached to one side of the nerve;
axons do not pass through the tumor
Consist of
Antoni -A areas of high cellularity
Nuclei form palisades Verocay bodies
Antoni -B myxoid areas
Schwannoma
Antoni A hyprecellular
Antoni B Sparsely cellular
2. Neurofibromas:
Benign tumors composed predominately of Schwann cells, but also
containing fibroblasts & perineural cells
May involve single or multiple dorsal spinal nerve roots (multiple in
patients with von Ricklinghausen's disease - NF1)
CN involvement is extremely rare
May present as
Localized fusiform enlargement of a nerve or
Extensively infiltrate along the nerve ropy enlargement of the
nerve & its branches (plexiform Neurofibroma)
Plexiform neurofibromas are usually part of NF1, excision is very
difficult
Histology:
Wavy spindle shaped cells, myxoid collagenous stroma with
interspersed nerve fibers
Acoustic Neurinoma
(Schwannoma)
METASTATIC ICTS
50% of ICTs.
Common Primaries:
Broncho-genic small cell undifferentiated (oat cell) ca.,
Breast ca., Malignant melanoma, RCC. & Colon ca.
Sites of metastases:
Cerebral cortex 80%;
Rest are in the cerebellum & brain stem.
50% are multiple; at the junction between the gray &
white matter.
Vertebral column is a common site for metastases
of
Breast & Prostatic carcinomas
Thoracic spine 60%, Cervical 20% & Lumbar 20%
Treatment : Radiotherapy
CHOROID PLEXUS PAPILLOMA
MC in children Arising from the lateral
ventricles
In adults they are found MC in the 4th
ventricle
Present with Hydrocephalus
Due to either over-production of CSF or to
obstruction of the ventricular system.
Consist of papillae with fibrovascular
stalks covered with a cuboidal or columnar
ciliated epithelium, recapitulating the
structure of the normal choroid plexus.
COLLOID CYST OF THE THIRD VENTRICLE
A non-neoplastic cystic lesion
Morphology:
Having a thin fibrous capsule, a lining of Cuboidal to
columnar epithelium & containing gelatinous
Proteinaceous material.
Attached to the roof of the third ventricle at the
foramina of Munroe & may cause sudden
obstruction of the CSF flow acute non-
communicating hydrocephalus brain
herniation & death
Symptoms: headaches (often positional), drop
attacks, incontinence
Goblet cells are confirmatory
Colloid cyst
MISCELLANEOUS (MIDLINE) TUMORS
Pinealomas:
True pineocytomas are extremely rare, may also have
pineoblastomas
Germinomas:
MC in the pineal & suprasellar regions in adolescents &
young adults
Closely resemble testicular Seminomas & ovarian
Dysgerminomas
Other GCTs (Teratomas & Choriocarcinomas) also occur
Not clear how GCTs arise within the CNS
Craniopharyngiomas:
Benign cystic tumors of children & adolescents
Develop in the suprasellar region Hypopituitarism
Originate from remnants of Rathkes pouch & contain
squamous & columnar epithelium, calcifications are
common.
CNS LYMPHOMA
Primary CNS lymphomas
Account for ~1% of ICTs
MC CNS neoplasm in AIDS & other
immunosuppressed patients often arise deep within
the cerebral hemispheres & are commonly bilateral
Lymphoma cells exhibit an angiocentric distribution
Usually are B-cell lymphomas & many appear to be
EBV-related.
Secondary CNS lymphomas
Lymphomas arising outside of the CNS rarely involve
the brain parenchyma
May involve the meninges, intradural spinal nerve
roots & epidural space
PHAKOMATOSES
NEUROCUTANEOUS SYNDROMES
(PHAKOMATOSES)
AD
Hamartomas & Neoplasms
Esp. involving the nervous system & skin
Mutations in tumor suppressor genes
1. Neurofibromatosis Type 1 (NF1):
Neurofibromas, Neurofibro-sarcomas
Optic nerve Gliomas
Pigmented cutaneous macules (caf au lait spots)
Pigmented nodules of iris (Lisch nodules)
2. Neurofibromatosis Type 2 (NF2):
Bilateral Schwannomas of CN VIII
Multiple meningiomas
Spinal cord Ependymomas
3. Tuberous Sclerosis
Hamartomas (tubers) in the cerebral
cortex, Sub-Ependymal hamartomas
(candle drippings) Sub-Ependymal
giant cell Astrocytomas
Seizures & mental retardation
Extra CNS findings:
Kidney (Angiomyolipoma), Heart
(Rhabdomyoma MCC in kids, adult =
mixomas ), skin (Angiofibroma)
Tuberous Sclerosis
4. von Hippel-Lindau disease
Hemangioblastomas of the cerebellum,
retina, brain stem & spinal cord
Cysts of liver, kidney & pancreas
incidence of RCC, may be bilateral
~ 10% of Hemangioblastomas
polycythemia
Diagnosis of brain tumors
Clinical
Laboratory
Radiological
Treatment

Colloid cyst
Pudenz
Principles of Treatment:
Surgery: initial treatment
Radiotherapy
Chemotherapy
Combine treatment
Approaches in brain surgery
Vault approaches
Craniotomy flap
Burr hole procedures
Endoscopic assisted
Endoscopic
Transcortical , extraaxial
Microscopic assisted

Skull bases approaches
Subfrontal
Pterional
Temporal ,subtemporal
Retrosigmoid
Far lateral
Petrosal
Transsphenoidal

Transcranial
Approach
Microscopic Transseptal-
transsphenoidal Pituitary Adenoma
Resection
Glioblastoma - Surgery
Stereotaxis

Radiotherapy
Conventional radiation - field
Stereotactic radiosurgery
- gamma knife Co 60
- linac SRS - single dose /
fractionation

Efficacy vs complications radionecrosis

Roles of Radiotherapy in Brain Tumor
Incomplete surgery
Deep seated tumor: midbrain, pons
Radiosensitive tumor: medulloblastoma
Pituitary adenoma
Palliation: metastatic lesions
Roles of Whole Cerebrospinal Axis
Irradiation:
Medulloblastoma
Ependymoma: esp. poorly differentiated
type
Malignant glioma of posterior fossa
Pinealoma
Choroid plexus papilloma
Reticulum cell sarcoma of the brian
Chemotherapy
BCNU
Temozolomide
Procarbazine
Cisplatin
Antiangiogenics / Thalidomide
Prognostic Factors: for malignant
astrocytomas
Age
Tumor type
Performance status
Extent of surgery