Primary brain tumors

Done by : Dana Hamo

Introduction
Brain tumors are masses of abnormal cells within the brain.
They can be primary or metastatic, benign or malignant.
Clinical features and radiological findings vary according to
the type, location, and onset of the tumor.
Magnetic resonance imaging (MRI) is the primary diagnostic
method.
The histological grade of the tumor is an important factor in
determining the prognosis.
Removal of the entire tumor is a prerequisite for remission.
Malignant tumors usually require additional treatment
with radiotherapy and/or chemotherapy.
Classification

 Primary brain tumors arise

within the CNS and are
classified based on the
growth characteristics and
cell type from which
the tumor arises.
By the spreading potential
 By the cell type
• Gliomas (e.g., astrocytomas and oligodendrogliomas)
• Choroid plexus tumors (e.g., choroid plexus papilloma, choroid plexus carcinoma)
• Neuronal and mixed neuronal-glial tumors (e.g., paraganglioma, central neurocytoma)
• Meningiomas (see “Classification of meningiomas”)
• Embryonal neuroectodermal tumors (e.g., medulloblastoma, CNS neuroblastoma)
• Pituitary tumors: a group of tumors that arise from the pituitary gland (e.g., pituitary adenoma, craniopharyngioma)
• Pineal tumors
• Tumors of the cranial and paraspinal nerves (e.g., schwannoma, neuroma, neurofibroma)
• Mesenchymal nonmeningeothelial tumors (e.g., hemangiomas, hemangioblastoma, lipoma, hamartoma)
• Germ cell tumors (e.g., teratoma)
• Primary central nervous system lymphoma (PCNSL)
 THE
GLIOMAS
These tumors arise from the neuroectoderm and form the majority (50%) of
brain tumors. They arise from glial supporting cells and grow to infiltrate brain
tissue making removal and treatment difficult.
Many factors have been incriminated in their formation like exposure to
radiation and petrochemicals.
They tend to occur mostly in males between the ages of 50 to 70 years.
However, some low-grade gliomas occur in children.
Gliomas are divided into several categories depending on their cell of origin,
so, we have astrocytomas, oligodendrogliomas and ependymomas.
 1. ASTROCYTOMA

• The most c o m m o n primary intra-axial brain tumor

• Astrocytoma's vary in terms of malignancy, from low grade to high grade.

• People c a n develop astrocytoma's at any age. The low-grade type is more

often found in children or young adults, while the high-grade type is more
prevalent in adults.
 GRADE 1 PILOCYTIC ASTROCYTOMA

• They are considered benign tumors, occurring mostly in children.

• Often associated with NF1.

• They usually present as cystic lesions in the cerebellum (ie infratentorial).

• They could occur in young adults in areas like the brain stem

• Pro gnosis: favo ra b le

• Survival rate >10 years, cure if total resection.

GRADE 1 PILOCYTIC ASTROCYTOMA
MRI h e a d (a: T1-weighted; with
contrast; sagittal plane; b: T2-
weighted; axial plane) of a child
A large lesion in the right cerebellar
hemisphere displaces the vermis to
the left of midline (T1 hypointense
a nd T2 hyperintense) a nd a
heterogeneously enhancing solid
nodular component (green overlay).

Cerebellar pilocytic astrocytoma

frequently manifests as a large cystic
lesion with a n enhancing mural
nodule.
MRI sequences from left T1, T2 and T1 with Gadolinium
showing a pilocytic astrocytoma compressing the fourth ventricle
and producing
hydrocephalus as indicated by the dilated right temporal horn.
 GRADE 2 DIFFUSE FIBRILLARY
ASTROCYTOMA
• They are slowly growing infiltrative tumors.

• They look like brain tissue with no apparent margin or capsule.

• Arises both in adults a n d children (cerebellum).

• Most commonly found in the frontal a n d temporal lobes.

• Incurable, survival approximately 5 years.

GRADE 2 DIFFUSE FIBRILLARY
ASTROCYTOMA
• A : c ontrast-e nhan ce d T1-
weighted image showing
a nonenhancing mass in
the left parietal lobe.
• B: The mass has relative ly
homogeneous increased
signal intensity in the T2-
weighted image.
 GRADE 3 ANAPLASTIC ASTROCYTOMA

• These tumors are infiltrative with rapid growth

• They are highly vascular tumors with areas of
necrosis a n d high mitotic figures.

• Incurable, survival 1.5-2 years.

GRADE 3 ANAPLASTIC ASTROCYTOMA

• (A) T1-weighted (B a n d C) T2-

weighted a n d (D)
postcontrast T1-weighted
axial as well as post-contrast
T2-weighted (E) sagittal a n d
(F) c o ro nal images revealed
a n ill-defined insular mass in
the right hemisphere.
 GRADE 4 GLIOBLASTOMA

• The most malignant type of astrocytoma

• The most c o m m o n malignant primary brain tumor

• Rapid growth, abrupt symptom onset, a n d short disease course

(death within weeks)

• They h ave necrotic areas with new vessel formation a n d areas of

hemorrhage may b e seen occasionally.

• In cura ble, surviva l 12 months, 10% at 2 ye a rs

GRADE 4 GLIOBLASTOMA
T1-weighted contrast-enhanced MRI,
frontal view: bihemisphere garland-like
contrast enhancement. There is peripheral
contrast agent enhancement (blue arrow)
with central necrosis (green arrow). The
third ventricle is centrally located. The
tumor is displacing the posterior horn of
the lateral ventricle (yellow overlay).
Diagnosis: bilateral glioblastoma (butterfly
glioma).
 MANAGEMENT
• Medically, steroids in tumor doses usually help to control edema, and anti-
epileptic drugs may be used to control the seizures if present.

• Many surgical techniques are used to help guide the neurosurgeon for proper
localization and excision. These include but not limited to, the use of surgical
microscopes, neuro-navigation, intraoperative ultrasound, intraoperative MRI and
the localization of tumor by injection of dyes taken by the tumor cells only.

• The aim of surgery is to make a proper histological diagnosis, remove or debulk

the tumor, reduce the effects of the tumor on the brain and reduce the dosage of
radiotherapy if given postoperatively to the malignant types.
 2. OLIGODENDROGLIOMA

• Tumor that arises from oligodendrocytes.

• Relatively rare, a n d slow growing tumor most often found in the frontal
lobes (cortical and subcortical).
• M e dian a g e is 40-50
• Due to being found in the frontal lobe it is often associated with seizures,
focal neurological deficits, a n d personality changes.
• O n imaging it appears as a n intra-parenchymal tumor with calcifications,
masses involving the cortex or subcortical white matter. (CT: hypodense,
T1:hypointense, T2:hyperintense )
• Biopsy: fried e g g cells (cells with a clear cytoplasm a n d round nucleus) ,chicken-wire pattern of capillary
anastomses
• Treated by resection a n d adjuvant radiotherapy a n d c h e m o therapy.
• Recurrence: nearly 100%
• 5-year survival rate 50-60%
• Non contrast
CT:
hypodense
lesion with
multiple
intralesional
hyperdense
calcifications is
visible in the
temporal lobe
Fried e g g cells
 3. Ependymomas

•Epidemiology: peak incidence in children and young adults

•Associated conditions: neurofibromatosis type II
•Clinical features: the 4th ventricle is the most common location in children
→ noncommunicating hydrocephalus → features of increased intracranial
pressure (e.g., papilledema, headache)
•Diagnostics

• Imaging: intra-parenchymal tumor with calcifications and cystic components due

to necrosis and/or hemorrhage
• Biopsy
• Perivascular pseudorosettes
• Rod-shaped bodies (blepharoplasts) near the nucleus
•Treatment

• Resection
• Adjuvant radiotherapy
•Prognosis

• Depends on the WHO grade, but usually poor

• Overall 5-year survival rate: 65–90%
Cranial MRI (FLAIR-sequence, sagittal view)
A hyperintense, heterogeneous, extended
mass is visible on the roof of the 4th
ventricle. At the superior aspect of the
tumor, multiple cysts are present. As a result
of an obstructed circulation of the
cerebrospinal fluid, the 3rd ventricle appears
widened.
Ependymal pseudorosette
 4. MEDULLOBLASTOMA
• Highly malignant primary brain tumor in children.
• Occurs in cerebellum often in midline (1.truncal ataxia if it involves cerebellar
vermis: inability to sit upright / stand without support, 2.cerebellar defects: broad-
based gait).

• More c o m m o n in males.

• C a n compress 4th ventricle → causing non-communicating hydrocephalus causing

h e a d a c h e a n d papilledema.
• C a n spread to CSF:
-Nodules in dura of spinal cord: “Drop metastasis”.
-Tend to occur in lower spinal cord, c a u d a equina syndrome.
-Back pain, focal neuro lesions c a n occur.
• Associated with Turcot syndrome (FAP that is associated with gliomas of Lynch
syndrome)
• Dia gn o sis:
Imaging: intraparenchymal contrast-enhancing mass (CT: iso/hyperdense, MRI T1 hypointense,
T2:isointense)
Histology: Homer-Wrights Rosettes. (small round blue cells surrounding a central neutrophil)

• Treatment:
Surgical resection, complementary Radiotherapy for the neuroaxis, a n d chemotherapy when
recurred.

• Prognosis:
50%-60%survival at 5 ye ars.
75%c h ildren survive to adulthood.
Many with complications of treatment.
 4. MENINGIOMA
• Most c o m m o n benign primary intracranial tumor in adults, arising from arachnoid
membrane (thus c a n arise in any part of the CNS covered by the meninges).

• Mostly idiopathic. Also due to ionizing radiation.

• NF2 is asso ciate d with the d e velopm e nt o f m ultiple m e ningiom as.

• This tumor is often calcified, may cause hyperostosis of adjacent bo ne (detectable on

imaging)

• Many are asymptomatic a n d c a n b e a n incidental finding. (if it developed symptoms, it

would b e the general symptoms of CNS tumors (eg seizure,..) ).
• It has a female predominance a n d has be e n tied to hormonal disturbances a n d to
trauma.
 MENIN G I O M A

• Investigations:
- CT scan with contrast (Hyperdense, homogenous lesion, with dural borders).

- Imaging modality of choice: MRI

- MRI with contrast (MRI will show a n isointense lesion on the T1 weighted
sequence, which enhances well with gadolinium).

- Both CT a n d MRI will show the vasogenic e d e m a which a c c o m p a n y

meningioma's.
•Olfactory groove meningioma on
(left) T1 M RI with c ontrast
(right) axial CT with contrast
 Pineal re gion
meningioma on T1
with c ontrast

• Foramen magnum meningioma

MRI:
Left: T1 with c ontrast
Right: T2
 MENIN G I O M A

• Management:
• - Conservative management (asymptomatic and/or non-progressive on CT/MRI).
• - Surgery resection (curative if complete resection a n d indicated when symptomatic
and/or documented growth on serial CT/MRI).

• - Endovascular embolization for highly vascularized, likely bloody, tumors to facilitate

surgery.

• - Radiation therapy (in c a s e of inoperable tumors, postop, as a n adjuvant, a n d in

small tumors).
5. VESTIBULAR SCHWANOMA

 Also known as acoustic neuroma, slow growing, benign, posterior fossa

tumor that arises from Schwann cells.
 Arises from vestibular nerve of CN VIII in internal auditory canal,
expanding into bony canal and cerebello-pontine angle (CPA).
 Characterized by unilateral progressive hearing loss, tinnitus,
disequilibrium/unsteady gait, and dizziness (compression of CN VIII).
 Late symptoms: due to pressure of adjacent structures (CN V & VII
involvement )
 If the tumor was bilateral, it is diagnostic of NF2.
VESTIBULAR SCHWANOMA

 Investigations:
 MRI with contrast(gadolinium or T2 FIESTA sequence (>98%
sensitive/specific)).
 CT with contrast as a second choice.
 CN testing:
 CN VIII: Pure tone audiometrytest, Weber & Rinne tests.
 CN V: ipsilateral decreased corneal reflex.
 CN VII: ipsilateral facial twitching or weakness.
• T1 weighted axial cranial
MRI
• In the first image, a
hyperintense lesion in the
cerebellopontine angle is
visible. In the second
image, ingrowth into the
internal auditory meatus
can be seen
Cranial MRI
Upper left: T2 weighted
image (axial view)
Lower left and right: T1
with contrast (axial and
coronal
views)
A tumor is visible on the
right side between the
brainstem
and the cerebellum. There
is strong contrast
enhancement
within the lesion and it has
a hypointense center,
suggesting
necrosis.
VESTIBULAR SCHWANOMA

 Management:
 Monitoring through serial imaging (CT/MRI) and audiometry if tumor is
small, hearing is still preserved.
 Surgical excision of the tumor.
 Radiation therapy.
 They have a good prognosis.
SUMMARY
 SUMMARY

 - Most common brain tumors ---> metastasis

 - Most common primary brain tumors ---> those of astrocytes (PCA vs GBM)
 - Most common benign brain tumor in adults ---> meningioma
 - Most common pediatric primary brain tumor ---> PCA
 - Most common calcifying brain tumors is ---> oligodendroglioma
 - Most common 4th ventricle- involving tumor ---> ependymoma
 - Exclusively common in female ---> meningioma
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