Brain tumors for medical

students
Abat Sahlu.Oct 23,18
 Tumors

• Brain tumors
- primary
- metastatic
• Spinal cord tumors
• Intradural - intramedullary – astrocytoma
- ependymomas
- extramedullary - meningioma
- schwannoma
• Extradural - metastatic
- primary
 Location, location, location…

CHILDREN
 ADULTS

Location, location, location…

 Overview of WHO classification of nervous
system tumors
1. Tumors of neuroepithelial tissue
2. Tumors of cranial and paraspinal nerves
3. Tumors of the meninges
4. Lymphomas and hematopoietic neoplasms
5. Germ cell tumors
6. Tumors of the sellar region
7. Metastatic tumors
 Classification

A. TUMORS OF NEUROEPITHELIAL TISSUE

B. TUMORS OF CRANIAL, SPINAL AND PERIPHERAL NERVES
C. TUMORS OF THE MENINGES
D. LYMPHOMAS AND HEMATOPOIETIC NEOPLASMS
E. GERM CELL TUMORS
F. TUMORS OF THE SELLAR REGION
G. METASTATIC TUMORS
H. LOCAL EXTENSIONS FROM REGIONAL TUMORS
I. CYSTS AND TUMOR-LIKE LESIONS
J. UNCLASSIFIED TUMORS
 A. TUMORS OF NEUROEPITHELIAL TISSUE

I. Astrocytes --astrocytic tumors

II. Oligodendrocytes-- oligodendroglial tumors
III. Oligoastrocytic tumors-- mixed gliomas
IV. Ependymocytes -- ependymal tumors
V. Choroid plexus tumors
VI. Other neuroepithelial tumors
VII. Neuronal and mixed neuronal-glial tumors
VIII. Pinealocytes →pineal parenchymal tumors
IX. Embryonal tumors
 Tumors of neuroepithelial tissue
I. Astrocytes astrocytic tumors
1. astrocytomas that are typically infiltrating (lower
grade tumors in this category tend to progress in
malignancy)
a. diffuse astrocytoma (WHO II)
b. anaplastic (malignant) astrocytoma (WHO III)
c. glioblastoma (WHO IV) (formerly glioblastoma
multiforme (GBM)).
d. gliomatosis cerebri
2. more circumscribed lesions (these do not
tend to progress to anaplastic astrocytoma and
GBM)
a. pilocytic astrocytoma
b. pleomorphic xanthoastrocytoma (PXA)
c. subependymal giant cell astocytoma
II. Oligodendrocytes oligodendroglial tumors
1. oligodendroglioma (WHO II)
2. anaplastic oligodendroglioma (WHO III)
Oligodendroglial tumors
● frequently presents with seizures
● predilection for the frontal lobes
● recommended treatment: surgery for mass
effect
Chemotherapy for all (with or without surgery),
XRT only for anaplastic transformation
III. Oligoastrocytic tumors ( mixed gliomas)

1. oligoastrocytoma
2. anaplastic (malignant) oligoastrocytoma
 V. Choroid plexus tumors

1. choroid plexus papilloma

2. atypical choroid plexus papilloma
3. choroid plexus carcinoma
VII. neuronal and mixed neuronal-glial tumors

1. gangliocytoma
2. ganglioglioma
3. central neurocytomas
VIII. Pinealocytes →pineal parenchymal
tumors
1. pineocytoma (pinealoma)
2. pineoblastoma
3. pineal parenchymal tumor of
intermediate differentiation
4. papillary tumor of the pineal region
 IX. Embryonal tumors

1. Medulloblastoma
2. CNS primitive neuroectodermal tumors(PNET)
a. CNS neuroblastoma
B. TUMORS OF CRANIAL, SPINAL AND PERIPHERAL NERVES

I. Schwannoma(vestibular schwannoma AKA

acoustic neuroma)
II. Neurofibroma
III. Perineurioma
IV. Malignant peripheral nerve sheath tumor
(MPNST) (neurogenic sarcoma, anaplastic
neurofibroma, “malignant schwannoma”).
 C. Tumors of the meninges

Tumors of meningothelial cells

1. meningioma .
a. meningothelial
b. fibrous (fibroblastic)
c. transitional (mixed)
II. Mesenchymal, non-meningothelial tumors
lipoma,
hemangioma
hemangiopericytoma
 Meningiomas

Meningiomas are the most common primary

intracranial tumors. They are usually slow
growing, extra-axial tumor, circumscribed(non-
infiltrating), benign lesions.
arise from arachnoid cap cells (not dura)
● most commonly located along falx, convexity,
or sphenoid bone
● most are cured if completely removed
 D. LYMPHOMAS AND HEMATOPOIETIC
NEOPLASMS

I. Malignant lymphoma(primary CNS lymphoma)

II. Plasmacytoma
III. Granulocytic sarcoma
 F. TUMORS OF THE SELLAR REGION

I. Craniopharyngioma—from embryonic tissue

II. Adenohypophyseal cells pituitary adenomas
1. prolactinoma
2. ACTH secreting adenoma
3. growth-hormone secreting adenoma
4. thyrotropin (TSH) secreting adenoma
5. gonadotropin (LH and/or FSH) secreting adenoma
III. Neurohypophysis and infundibulum
IV. Pituitary carcinoma
 E. GERM CELL TUMORS

I. Germinoma
II. Embryonal carcinoma
III. Endodermal sinus tumor (EST) (yolk sac
tumor)
IV. Choriocarcinoma
V. Teratoma (from all 3 germ-cell layers)
VI. Mixed germ cell tumors
 G. METASTATIC TUMORS

Those commonly involving brain include:

I. Lung cancer: especially small-cell
II. Breast
III. Melanoma
IV. Renal cell
V. Lymphoma
VI. GI
 H. LOCAL EXTENSIONS FROM
REGIONAL TUMORS
I. Paraganglioma (chemodectoma)
Glomus jugulare tumor
II. Notochord chordoma
III. Carcinoma
 I. CYSTS AND TUMOR-LIKE LESIONS

Rathke’s cleft cysts

Epidermoid cyst
Dermoid cyst
Colloid cyst of the third ventricle
Neuroglial cysts
J. UNCLASSIFIED TUMORS
 Brain tumors – general clinical aspects
General information

Brain tumors present with:

● Progressive neurologic deficit usually motor
weakness
● Headache:
● Seizures Often focal in onset (due to cortical
irritation in the area of the tumor), may
generalize secondarily.
 Focal neurologic deficits associated with brain tumors

• In addition to nonfocal signs and symptoms

(e.g. seizures, increased ICP…), as with any
destructive brain lesion, tumors may produce
progressive deficits related to the function of
the involved brain. Some characteristic
“syndromes”:
• 1. frontal lobe: dementia, personality changes.
Often non lateralizing, but hemiparesis or
dysphasia (with dominant hemisphere involvement)
may occur
• 2. temporal lobe: auditory or olfactory
hallucinations, memory impairment.
3. parietal lobe:
4. occipital lobe: contralateral visual field deficits.
5. posterior fossa: cranial nerve deficits, ataxia
(truncal or appendicular)
 Pituitary Tumors – General Information and Classification

 Microadenoma: A pituitary y tumor < 1 cm diameter.

 Macroadenomas: Tumors >1 cm diameter.
 most are benign
 hormonal effects (hyperprolactinemia, Cushing’s
syndrome, acromegaly…),
 Mass effect (most commonly: bitemporal
hemianopsia from compression of optic chiasm),
 As an incidental finding,
 infrequently with pituitary apoplexy
● prolactinoma medical therapy (DA agonists) .
For other tumor types, options primarily consist
of surgery (transsphenoidal or transcranial), or
XRT
● post-op concerns include: diabetes insipidus,
CSF leak
 Supratentorial tumors
Signs and symptoms include

1. those due to increased ICP :

a) from mass effect of tumor and/or edema
b) from blockage of CSF drainage
(hydrocephalus): less common in supratentorial
tumors (classically occurs with colloid cyst, may
also occur with entrapped lateral ventricle)
2. progressive focal deficits: includes weakness,
dysphasia
a) due to destruction of brain parenchyma by tumor
invasion
b) due to compression of brain parenchyma by mass
and/or peritumoral edema and/or hemorrhage
c) due to compression of cranial nerve(s)
3. headache:
4. Seizures. Rare with posterior fossa tumors or
pituitary tumors
5. mental status changes: depression, lethargy,
apathy, confusion
6. symptoms suggestive of a TIA or stroke, may
be due to:
a) occlusion of a vessel by tumor cells
b) hemorrhage into the tumor: any tumor may
hemorrhage,
c) focal seizure
7. in the special case of pituitary tumors
a) symptoms due to endocrine disturbances
b) pituitary apoplexy
c) CSF leak
 Infratentorial tumors
Signs and symptoms

• most posterior fossa tumors present with signs and symptoms of

increased intracranial pressure (ICP) due to hydrocephalus (HCP).
include:
a) headache:
b) nausea/vomiting: due either to increased ICP from HCP, or from
direct pressure on the vagal nucleus or the area postrema (so-called
“vomiting center”)
c) papilledema:(more common when the tumor impairs CSF circulation)
d) gait disturbance/ataxia
e) vertigo
f) diplopia: may be due to VI nerve (abducens) palsy which may occur
with increased ICP in the absence of direct compression of the nerve
2. S/S indicative of mass effect in various locations
within the p-fossa
a) lesions in cerebellar hemisphere may cause: ataxia
of the extremities, dysmetria, intention tremor
b) lesions of cerebellar vermis may cause: broad
based gait, truncal ataxia
c) brainstem involvement usually results in multiple
cranial nerve and long tract abnormalities, and should
be suspected when nystagmus is present (especially
rotatory or vertical)
 Pediatric brain tumors

Among all childhood cancers, brain tumors are

the second only to leukemias
are the most common solid pediatric tumor,
comprising 40–50% of all tumors.
• Almost all patients have hydrocephalus by the
time of presentation , causing typical signs and
symptoms of headache, vomiting, lethargy,
memory disturbance
• Drop metastases from CSF seeding can
produce radiculopathy and/or myelopathy.
 The common pediatric brain tumors

• Medulloblastoma
• cerebellar astrocytoma
• Ependymomas
• pineal tumors,
• craniopharyngiomas,
• Many of these tumors escape diagnosis until they
are very large in size due to the elasticity of the
infant skull, the adaptability of the developing
nervous system to compensate for deficits, and the
difficulty in examining a patient and inability to
cooperate.
• The most common presenting manifestations are
vomiting, arrest or regression of psychomotor
development, macrocrania, poor feeding/failure to
thrive. They may also present with seizures.
 Medications for brain tumors

• Dexamethasone The beneficial effect of

steroids in metastatic tumors is often much
more dramatic than with primary infiltrating
gliomas.
• Antiepileptic drugs (AEDs)
• Chemotherapy Temozolomide
 Tumor markers used clinically

• Human chorionic gonadotropin (hCG)

• Alpha-fetoprotein
• Carcinoembryonic antigen (CEA)
• S-100 protein
 Tumors of the Spine and Spinal Cord

• 15% of primary CNS tumors are intra spinal

• Most primary CNS spinal tumors are benign
(unlike the case with intracranial tumors).
• Most present by compression rather than
invasion.
 Extradural spinal cord tumors (55%)

Arise in vertebral bodies or epidural tissues

1. metastatic: comprise the majority of ED tumors
a) most are osteolytic (cause bony destruction):
● lymphoma
● lung
● breast
● prostate
b) metastases that may be osteoblastic:
● in men: prostate Ca is the most common
● in women: breast Ca is the most common
• 2. primary spinal tumors (very rare)
• a) chordomas
• b) osteoid osteoma
• 2. Intradural extramedullary (ID-EM) arise in
leptomeninges or roots.
• meningiomas
• Neurofibromas/ Shwannomas
• 3. Intramedullary spinal cord tumors (IMSCT):
arise in SC substance. Invade and destroy
tracts and grey matter
• Astrocytomas
• Ependymomas
 Investigations
• Basic lab
• CT
• MRI
• ANGIOGRAPHY
 RX
• Dexamethasone
• Surgical
REFERENCE