Intracranial TumorsIntracranial Tumors

Brain TumorsBrain TumorsBrain TumorsBrain Tumors

Primary brain tumors occur in 6 persons per 100 000 per year.

In adults 80-85% of the brain tumors localized in supratentorial region 15-20% of the brain tumors localized in infratentorial region

Presentation: progressive neurologic deficit (68%) Headache (54%) Seizures (26%)

Presentation of Supratentorial TumorsPresentation of Supratentorial Tumors

Increased ICP Progressive focal deficits Headache Seizures Mental status changes Symptoms suggestive of a TIA or stroke Endocrine disturbances

Presentation of posterior fossa tumorsPresentation of posterior fossa tumors

Increased ICP due to hydrocephalus. These include headache, nausea/ vomiting, papilledema, ataxia, vertigo, diplopia.

Lesions in cerebellum may cause ataxia, dysmetria, and intention tremor.

Brain stem involvement usually results in multiple cranial nerve and long tract abnormalities

WHO Classification of CNS Tumors (2007)WHO Classification of CNS Tumors (2007)

1 Tumors of neuroepithelial tissue 2 Tumors of cranial and paraspinal nerves 3 Tumors of meninges 4 Lymphomas and hemopoietic neoplasms 5 Germ cell tumors 6 Tumors of the sellar region 7 Metastatic tumors

11 Tumors of Neuroepithelial TissueTumors of Neuroepithelial Tissue 1.1 ASTROCYTIC TUMORS

Pilocytic Astrocytoma (Grade I) Diffuse Astrocytoma (Grade II) Anaplastic Astrocytoma (Grade III) Glioblastoma (Grade IV)

1.2. OLIGODENDROGLIAL TUMORS Oligodendroglioma (Grade II) Anaplastic Oligodendroglioma (Grade III)

1.4. EPENDYMAL TUMORS Ependymoma (Grade II)

11 Tumors of Neuroepithelial TissueTumors of Neuroepithelial Tissue

1.5 CHOROID PLEXUS TUMORS Choroid Plexus Papilloma (Grade I)

1.8. PINEAL PARENCHYMAL TUMORS Pineocytoma (Grade I) Pineoblastoma (Grade IV)

1.9. EMBRYONAL TUMORS Medulloblastoma (Grade IV)

1.1 Astrocytic Tumors(most common primary intra-axial brain tumors)

Pilocytic Astrocytoma (Grade I): slow growing benign tumor location:

• optic gliomas & hypothalamic gliomas • brainstem gliomas • cerebellum • spinal cord

treatment of choice is surgical excision

1.1 ASTROCYTIC TUMORS

Diffuse Astrocytoma (Grade II) tend to occur in children and young adults demonstrate low degrees of cellularity and preservation of

normal brain elements within the tumor surgery is the principal treatment radiation is not indicated

1.1 Astrocytic Tumors

Anaplastic Astrocytoma (Grade III): occur in patients who are generally a decade older than

those with an astrocytoma but a decade younger than those with a glioblastoma

duration of symptoms is usually shorter than that for the former lesion and longer than that for the latter

surgery followed by radiotherapy has become the standard

re-operation for recurrence extends survival

1.1 Astrocytic Tumors

Glioblastoma (Grade IV): the commonest primary brain tumor. It is also the most malignant of

astrocytic tumors. It occurs most commons in the cerebral hemisphere in the fifth

decade or above the symptoms of glioblastoma are usually of recent onset, and the

patient is more ill it has characteristic ring of contrast enhancement surrounds a

center of low density, and has a zone of low density or edema around the ring enhancement

untreated glioblastomas is one of rapid progression radical surgery followed by radiotherapy + chemotherapy is

recommended re-operation for recurrence extends survival

1.2. Oligodendroglial Tumors

Oligodendroglioma (Grade II): frequently presents with seizures often calcified, predilection for the frontal lobes comprises 4% of primary brain tumor aggressive removal leads to longer survival.

Anaplastic Oligodendroglioma (Grade III): surgery followed by chemotherapy is recommended

1.4. Ependymal Tumors Ependymoma (Grade II):

5-6% of intracranial gliomas, mostly occurs in children usually well circumscribed and benign, commonly arises

in the floor of the fourth ventricle comprises 25% of tumors in region of 4th ventricles has potential to spread through CSF to spinal cord, a

process known as seeding mostly those of posterior fossa mass with increased ICP

(headache, nausea and vomiting), ataxia or vertigo goal of surgery is maximal possible resection without

causing neurological deficits total surgical resection of followed by craniospinal

radiotherapy is recommended

1.5 Choroid Plexus Tumors

Choroid Plexus Papilloma (Grade I): 0.4-1% of all intracranial tumors most of patients are <2 years old it is histologically benign tumor may become symptomatic because of the increased ICP

produced by secretion of CSF maybe cured surgically with total removal

1.7 Neuronal and Mixed Neuronal-Glial Tumors

Paraganglioma (Chemodectoma, Glomus tumors) histologically benign and slow growing tumors arises from paraganglion cells, usually in the area of the jugular

bulb, and tracks along vessels a very vascular tumor with main feeders from ECA patients commonly present with hearing loss and pulsatile

tinnitus combinations of palsies of cranial nerves IX, X, XI & XII occur

with occasional VII palsy radical surgical removal is recommended

1.8. Pineal Parenchymal Tumors

Pineocytoma (Grade I): a well-differentiated neoplasm arising from pineal epithelium it can metastasize through the CSF and is radiosensitive

Pineoblastoma (Grade IV): a malignant neoplasm arising from pineal epithelium it can metastasize through the CSF, and is radiosensitive

1.9. Embryonal Tumors Medulloblastoma (Grade IV):

15-20% of intracranial tumors in children, and is the most common malignant pediatric brain tumor

usually arises in the cerebellar vermis and predisposes to early obstructive hydrocephalus

usual presenting symptoms: headache, nausea and vomiting, ataxia

usually appears as a solid, midline, and IV-contrast-enhancing lesion on MRI

may disseminate via the CSF spontaneously or iatrogenically following surgery

prophylactic craniospinal radiotherapy is indicated following surgical removal

in children radiotherapy causes intellectual impairment and growth retardation and is avoided before 3 years of age

2 Tumors of Cranial and Paraspinal Nerves

2.1 SCHWANNOMA (Neurinoma) (Grade I) slow growing, benign tumor most arise from superior vestibular division of 8th nerve 8-10% of intracranial tumors incidence is 1 case per 100.000 population 95% are unilateral bilateral tumors being pathognomonic of NF2 the incidence of VS is increased in NF2 most common early symptoms are unilateral progressive hearing loss

and tinnitus otalgia, facial numbness and weakness, and taste changes occur as

the tumor enlarges larger tumors cause brain stem compression with ataxia, nausea and

vomiting, diplopia, cerebellar signs, and lower cranial nerve palsies

2 Tumors of Cranial and Paraspinal Nerves

2.1 SCHWANNOMA (Neurinoma) (Grade I) MRI is the diagnostic procedure of choice round or oval enhancing tumor centered on internal acoustic meatus all patients should have the detailed audiologic exam total surgical excision is the treatment of choice for most tumors for small tumors follow symptoms, hearing, and tumor size by serial

MRI intervention is recommended if growth > 2mm/year total excision of tumor is the goal of surgery facial nerve is pushed forward by the tumor and preservation of the

facial nerve is very important facial re-animation (hypoglossal-facial anastomosis) is performed

after 1-2 months if VII was divided, or if no function returns after 1 year with an anatomically intact nerve

3 Tumors of the Meninges

3.1 TUMORS of MENINGOTHELIAL CELLS Meningiomas:

15% of primary intracranial tumors slow growing, extra-axial circumscribed, usually benign tumors, which

arise from arachnoid (not dura) frequently calcified and often cause hyperostosis of adjacent bone usually cured if completely removed most commonly located along the parasagittal and falx, convexity, or

sphenoid bone, however may occur anywhere that arachnoid cells are found

they can be asymptomatic may be multiple in some of cases, this finding is more common in

neurofibromatosis

3 Tumors of the Meninges

3.1 TUMORS of MENINGOTHELIAL CELLS Meningiomas:

peak incidence at 45 years age, rarely occur in childhood and adolescence

occur with a higher incidence among women than among men parasagittal and falx meningiomas up to half invade the superior

sagittal sinus plain x-rays may show calcifications within the tumor (in 10%),

hyperostosis of the skull, enlargement of vascular grooves (especially middle meningeal artery)

MRI, appear as homogeneous, densely enhancing mass with broad base of attachment along dural border and dural tail is a common finding

3 Tumors of the Meninges

3.1 TUMORS of MENINGOTHELIAL CELLS Meningiomas:

characteristically, it has external carotid artery feeders in angiography. Angiography also gives information about occlusion of venous sinuses

surgery is the treatment of choice for symptomatic meningiomas incidental meningiomas may be managed expectantly with serial

imaging, as meningiomas tend to grow slowly extent of surgical tumor removal is the most important factor in the

prevention of recurrence radiotherapy reserve for malignant (invasive), aggressive, or non-

resectable meningiomas

3 Tumors of the Meninges

3.4 OTHER NEOPLASMS RELATED to MENINGES Hemangioblastoma:

• it represents 1-2.5% of intracranial tumors• the most common primary intra-axial tumor in the adult posterior

fossa• 20% occur as part of von Lippel-Lindau disease • it is histologically benign tumor• mostly cystic with a mural nodule and nodules are very vascular• surgical treatment may be curative

4 Lymphomas and Hemopoietic Neoplasms

4.1. MALIGNANT LYMPHOMAS occur at any age most frequently arise in the cerebral hemispheres, where, as contrast-

enhancing masses lesions usually enhance homogeneously without the typical ring of the

glioblastoma CNS involvement with lymphoma may occur secondarily from systemic

lymphoma, or may arise primarily in the CNS 10-30% of patients has multiple lesions there is an almost diagnostic tendency of rapid partial to complete resolution

on MRI following the administration of steroids, producing the so-called ghost tumor

the standard treatment after tissue biopsy is steroid therapy, chemotherapy and whole-brain radiation therapy

with no treatment, median survival is 2-3 months following diagnosis 80% of cases recur, usually 15 months after treatment

5 Germ Cell Tumors occur principally in the pineal and suprasellar regions almost all patients have hydrocephalus by the time of presentation aside from benign teratoma, all intracranial GCTs are malignant

and may metastasize via CSF and systemically characteristically (but not always) give rise to tumor markers

(ßhCG and AFP) in the CSF germinomas are malignant tumors of primitive germ cells that

occur in the gonads or in the CNS survival with these is much better than with non-germinomas

(embryonal carcinoma, choriocarcinoma, teratoma) some of the pure germinomas are potentially curable with radiation

therapy, but this is less likely for the other lesions, particularly the teratocarcinoma and choriocarcinoma

6 Tumors of the Sellar Region 6.1 CRANIOPHARYNGIOMA

benign tumors but difficulty in cure makes them malignant in behavior 2.5-4% of all brain tumors tend to arise from the pituitary stalk almost all CP have solid and cystic components and calcification is very

common surgical approach is usually via pterional, or combined (transcallosal

and pterional) diabetes insipidus often develops postoperatively postoperative mortality is 5-10% in most series, most from hypothalamic

injury post-op radiation probably helps prevent regrowth when residual tumor

is left behind, however, in pediatric cases it may be best to postpone radiotherapy, recognizing that re-operation may be necessary for recurrence

morbidity/mortality is higher with re-operation

7 Metastatic Tumors

most common sources of cerebral mets in adults are lung, breast, kidney

small cell Ca of the lung is more likely to produce cerebral mets the highest incidence of parenchymal mets is located on the

territory of MCA solitary lesion in the posterior fossa of an adult is considered a

metastasis until proven otherwise usually appear as well circumscribed non-complicated masses

on MRI usually enhance in MR, and must be considered in the

differential diagnosis of a ring-enhancing lesion with optimal treatment, median survival is only 32 weeks,

therefore management is mostly palliative

7 Metastatic Tumors

medical management with radiation therapy without surgery is recommended for patients with multiple cerebral metastases, or widespread systemic cancer and obviously short life expectancy

initial treatment: anticonvulsants, corticosteroids excisional surgery for solitary lesions is indicated when lesion

accessible, lesion is symptomatic or life-threatening, primary tumor known to be relatively radioresistant, or diagnosis unknown

stereotactic biopsy maybe considered far deep lesions, in patients who are poor surgical candidates, to ascertain a diagnosis when another diagnosis is possible, or for multiple small lesions

radiation therapy is usually administered following surgery, where micrometastases are presumed to be present throughout brain

Pituitary Adenoma

arise primarily from the anterior part of the pituitary gland represent 10% of intracranial tumors most common in the 3rd and 4th decades of life, and equally affect

both sexes usually present either due to endocrinologic disturbance, or due to

mass effect divided into two groups: functional (secreting), and non-functional

(non-secretory) functional tumors frequently present earlier with symptoms caused by

endocrinologic disturbances non-functional tumors usually do not present until of sufficient size to

cause neurologic deficits by mass effect

Pituitary Adenoma

panhypopituitarism may be caused by large tumors of the non-functional type

in large tumors optic chiasm may compressed (classically resulting in bitemporal hemianopsia)

in endocrinologic evaluation we have to check the adrenal axis, thyroid axis, gonadal axis, prolactin level, growth hormone, and neurohypophysis

lateral skull x-ray may help define anatomy of sphenoid sinus in cases where transsphenoidal surgery is planned

MRI is choice for pituitary adenomas microadenoma (< 1 cm), macroadenoma (> 1 cm)

Pituitary Adenoma

Prolactinoma: causes amenorrhea-galactorrhea syndrome in females,

impotence in males, and often infertility in either sex if no acute progression, these tumors may shrink dramatically

on bromocriptine (dopamine agonist) surgery is indicated if PRL >500 and tumor not controlled on

bromocriptine surgery followed by reinstitution of bromocriptine therapy may

normalize PRL

Pituitary Adenoma

Acromegaly: GH secreting tumors that cannot be managed medically causes acromegaly in adults, and gigantism in prepubertal

children if no contraindications, transsphenoidal surgery is the best initial

therapy for patients not cured by surgery, or with recurrences after

surgery, radiosurgery is indicated

Pituitary Adenoma

Cushing’s disease: elevated ACTH causes endogenous hypercortisolism

(Cushing’s disease) long-term efficacy of medical therapy is inadequate surgery is recommended

Pituitary Adenoma

transsphenoidal approach usually the procedure of choice (an extra-arachnoid approach, no external scar, and no brain retraction)

transsphenoidal approach indicated for microadenomas, macroadenomas without significant extension laterally beyond the confines of the sella and tumors with extension into sphenoid sinus

transcranial approaches (pterional) is useful for makroadenomas

Pituitary Adenoma

radiation therapy should not be routinely used following surgical removal of pituitary adenomas, follow patient with yearly MRI

treat recurrence with repeat operation consider radiosurgery if recurrence cannot be removed and mass

continues to grow

Pituitary Adenoma

Pituitary apoplexy onset of neurologic deterioration usually consisting of

headache, visual deficit, ophthalmoplegia, and reduced mental status

usually due to an expanding mass within the sella turcica as a result of hemorrhage, necrosis, or infarction within the tumor

CT or MRI shows hemorrhagic mass in sella and/or suprasellar region

emergency decompression to preserve vision is usually indicated

Chordoma

CHORDOMA benign, slow growing, locally aggressive, and have a high

recurrence rate rare tumors of the remnant of the primitive notochord. may arise at end of the notochord in the spheno-occipital

region (clivus) radical surgical removal is recommended

Cysts and Tumor-like Lesions

EPIDERMOID CYST occur most frequently in the leptomeninges, especially those

at the CPA and suprasellar region comprise 1% of intracranial tumors, and 7% of CPA tumors tend to spread along normal cleavage planes and surround

vital structures surgery is the essential treatment method

Cysts and Tumor-like Lesions

COLLOID CYST occurs in the anterior 3rd ventricle slow-growing benign tumor, and blocking foramina of Monro

causing hydrocephalus 0.5-1% of all intracranial tumors acute blockage of CSF can cause sudden death surgical removal is recommended