Brain tumor

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Assosciate editor(s)-In-Chief Fahimeh Shojaei, M.D., Prashanth Saddala, Sujit Routray, M.D. [2]

Synonyms and keywords: Brain tumour; brain cancer; intracranial tumor; astrocytoma; glioblastoma; oligodendrocytoma; meningioma; ependymoma; craniopharyngioma.

Overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Overview

Primary brain tumors include any tumor that starts in the brain. Primary brain tumors can start from brain cells, meninges, nerves, or glands. Brain tumors can be classified into two main categories: primary and secondary tumors. Primary tumors originate in astrocytes, oligodendrocytes and ependymal cells. Secondary tumors originate in malignant cancers located primarily in other organs. The cause of brain tumors is unknown. These cancers should be differentiated from essential tremor, stroke, chronic subdural hematoma, meningitis, orbital optic neuritis, arteriovenous malformations and brain abscesses. Radiation is one the common risk factors for brain tumors. The complications of brain tumors are brain herniation, loss of brain function and recurrence. The most common symptoms of brain tumors are headache, seizures, visual changes and changes in personality, mood and concentration. On head CT scan, brain cancer is characterized by hypodense mass lesions. On MRI brain, brain tumor is characterized by hypointense or isointense on T1-weighted scans, or hyperintense on T2-weighted MRI. The predominant therapy for brain tumor is surgical resection. Meningiomas, with the exception of some tumors located at the skull base, can be successfully removed surgically. In more difficult cases, stereotactic radiosurgery, such as Gamma Knife radiosurgery remains a viable option. Most pituitary adenomas can be removed surgically using a minimally invasive approach through the nasal cavity and skull base (trans-nasal, trans-sphenoidal approach). Larger pituitary adenomas require a craniotomy (opening of the skull) for their removal. Radiotherapy, including stereotactic approaches, is reserved for inoperable cases. Adjunctive chemotherapy and radiation may be required.

Historical Perspective

Classification

Brain tumors can be classified into two main categories: primary and secondary tumors. Primary tumors originate in astrocytes, oligodendrocytes and ependymal cells. Secondary tumors originate in malignant cancers located primarily in other organs. Brain tumors are classified depending on location of the tumor, type of tissue involved and whether they are benign or malignant.

Pathophysiology

Causes

The cause of brain tumors is unknown.^[1]

Differentiating brain tumors from other diseases

Brain tumors should be differentiated from essential tremor, stroke, chronic subdural hematoma, meningitis, orbital optic neuritis, arteriovenous malformation, intraocular optic neuritis and brain abscess.

Epidemiology and Demographics

In 2012, the prevalence and incidence of brain tumors were 148,818 and 6.4 per 100,000 persons per year respectively. The case-mortality rate of brain tumors is 4.4 per 100,000 persons per year. Patients of all age-group develop this cancer. Males are more affected with gliomas than females. Females are more affected with meningiomas. It usually affects individuals of the caucasian race. African Americans are less likely to develop this cancer.

Risk factors

Brain tumors are associated with a number of predisposing risk factors such as radiation, inherited conditions like neurofibromatosis; von Hippel-Lindau syndrome; Li-Fraumeni syndrome; Turcot syndrome, viruses such as Ebstein-Barr virus, chemicals such as formaldehyde; vinyl chloride; acrylnitrile and genetic factors.

Natural History, Complications and Prognosis

The complications of brain tumors are brain herniation, loss of brain function and recurrence. The prognosis in primary brain tumors depends on the type of tumor, age, functional status of the patient, the extent of surgical tumor removal, spread of metastasis and biogenetic markers.^[2]. Patients with benign gliomas may survive for many years^[3]^[4] while survival in most cases of glioblastoma multiforme is limited to a few months after diagnosis. The 5-year survival rate is 33.3%.

Diagnosis

Staging and Grading

The subtypes of brain tumors have different staging systems based on the grade, extent of spread, size and presence of distant metastases. The grades of brain tumors are subdivided into Grades 1,2,3 and 4. The lower the grade, better the prognosis.

History and Symptoms

The most common symptoms of brain tumors are headache, seizures, visual changes and changes in personality, mood and concentration.

Physical examination

Common physical examination findings of brain tumors include altered sense of consciousness, anisocoria, papilledema and focal neurological deficits.

CT

Imaging plays a central role in the diagnosis of brain tumors. On head CT scan, brain cancer is characterized by hypodense mass lesions.

MRI

Imaging plays a central role in the diagnosis of brain tumors. On MRI brain, brain tumor is characterized by hypointense or isointense on T1-weighted scans, or hyperintense on T2-weighted MRI.

Other imaging findings

The other imaging modalities that can be used for brain tumors are single-photon emission computed tomography, positron emission tomography and whole bone scan.

Other Diagnostic Studies

Electrophysiological exams, such as electroencephalography play a marginal role in the diagnosis of brain tumors. The definitive diagnosis of brain tumor can only be confirmed by histological examination of tumor tissue samples obtained either by means of brain biopsy or open surgery. The histologic examination is essential for determining the appropriate treatment and the correct prognosis.

Treatment

Medical Therapy

The predominant therapy for brain tumor is surgical resection. Adjunctive chemotherapy and radiation may be required.

Surgery

Meningiomas, with the exception of some tumors located at the skull base, can be successfully removed surgically. In more difficult cases, stereotactic radiosurgery, such as Gamma Knife radiosurgery, remains a viable option. Most pituitary adenomas can be removed surgically using a minimally invasive approach through the nasal cavity and skull base (trans-nasal, trans-sphenoidal approach). Larger pituitary adenomas require a craniotomy (opening of the skull) for their removal. Radiotherapy, including stereotactic approaches, is reserved for inoperable cases.

References

↑ National Library of Medicine.http://www.nlm.nih.gov/medlineplus/cancer.html
↑ Nicolato A, Gerosa MA, Fina P, Iuzzolino P, Giorgiutti F, Bricolo A. Prognostic factors in low-grade supratentorial astrocytomas: a uni-multivariate statistical analysis in 76 surgically treated adult patients. Surg Neurol 1995;44:208-21; discussion 221-3. PMID 8545771.
↑ Janny P, Cure H, Mohr M, Heldt N, Kwiatkowski F, Lemaire JJ, Plagne R, Rozan R. Low grade supratentorial astrocytomas. Management and prognostic factors. Cancer 1994;73:1937-45. PMID 8137221.
↑ Piepmeier J, Christopher S, Spencer D, Byrne T, Kim J, Knisel JP, Lacy J, Tsukerman L, Makuch R. Variations in the natural history and survival of patients with supratentorial low-grade astrocytomas. Neurosurgery 1996;38:872-8; discussion 878-9. PMID 8727811.

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Classification

Brain tumors can be classified as follows:

Brain tumors

Adult primary brain tumor

Childhood primary brain tumor

Brain metastase

•Glioblastoma multiforme
•Oligodendroglioma
•Meningioma
•Hemangioblastoma
•Pituitary adenoma
•Schwannoma
•Primary CNS lymphoma

•Pilocytic astrocytoma
•Medulloblastoma
•Ependymoma
•Craniopharyngioma
•Pinealoma

•Lung cancer
•Breast cancer
•Melanoma
•Gastrointestinal tract cancer
•Renal cell carcinoma
•Osteosarcoma
•Head and neck cancer
•Neuroblastoma
•Lymphoma
•Prostate cancer

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Overview

Brain tumors can be classified into two main categories: primary and secondary tumors. Primary tumors originate in astrocytes, oligodendrocytes and ependymal cells. Secondary tumors originate in malignant cancers located primarily in other organs. Brain tumors are classified depending on location of the tumor, type of tissue involved and whether they are benign or malignant.

Classification

Primary tumors

Tumors occurring in the brain include:

Most primary brain tumors originate from glia (gliomas) such as astrocytes (astrocytomas), oligodendrocytes (oligodendrogliomas), or ependymal cells (ependymoma). There are also mixed forms, with both an astrocytic and an oligodendroglial cell component. These are called mixed gliomas or oligoastrocytomas. Plus, mixed glio-neuronal tumors (tumors displaying a neuronal, as well as a glial component, e.g. gangliogliomas, disembryoplastic neuroepithelial tumors) and tumors originating from neuronal cells (e.g. gangliocytoma, central gangliocytoma) can also be encountered.

Other origins of primary brain tumors include: primitive neuroectodermal tumors (PNET, e.g. medulloblastoma, medulloepithelioma, neuroblastoma, retinoblastoma, ependymoblastoma), tumors of the pineal parenchyma (e.g. pineocytoma, pineoblastoma), ependymal cell tumors, choroid plexus tumors, neuroepithelial tumors of uncertain origin (e.g. gliomatosis cerebri, astroblastoma), etc.

Secondary tumors and non-tumor lesions

Secondary or metastatic brain tumors originate from malignant tumors (cancers) located primarily in other organs. Their incidence is higher than that of primary brain tumors. These tumor cells reach the brain via the blood-stream. The most frequent types of metastatic brain tumors are:

Some non-tumoral masses and lesions can mimic tumors of the central nervous system. These include:

Tuberculosis of the brain
Cerebral abscess (commonly in toxoplasmosis)
Hamartomas (for example, in tuberous sclerosis)
Von Recklinghausen neurofibromatosis

References

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Causes

The exact cause of brain tumor is not known.

. Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

The cause of primary brain tumors is unknown.^[1]

Cause

The cause of primary brain tumors is unknown.^[1]

References

↑ ^1.0 ^1.1 National Library of Medicine.http://www.nlm.nih.gov/medlineplus/cancer.html

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Differentiating Brain Tumor from other Diseases

On the basis of seizure, visual disturbance, and constitutional symptoms, brain tumors must be differentiated from AV malformation, brain aneurysm, bacterial brain abscess, tuberculosis, toxoplasmosis, hydatid cyst, CNS cryptococcosis, CNS aspergillosis, and brain metastasis.

	Symptoms				Physical examination	Lab Findings	MRI	Immunohistopathology
Diseases	Clinical manifestations					Para-clinical findings			Gold standard	Additional findings
	Symptoms				Physical examination	Para-clinical findings
	Head- ache	Seizure	Visual disturbance	Constitutional	Focal neurological deficit	Lab Findings	MRI	Immunohistopathology
Adult primary brain tumors
Glioblastoma multiforme	+	+/−	+/−	−	+	−	Supratentorial Irregular ring-nodular enhancing lesions Central necrosis Surrounding vasogenic edema Cross corpus callosum (butterfly glioma)	Astrocyte origin Pleomorphic cell Pseudopalisading appearance GFAP + Necrosis + Hemorrhage + Vascular prolifration +	Biopsy	Highest incidence in fifth and sixth decades of life Most of the time, focal neurological deficit is the presenting sign.
Oligodendroglioma	+	+	+/−	−	+	−	Almost always in cerebral hemisphers (frontal lobes) Hypointense on T1 Hyperintense on T2 Calcification Chicken wire capillary pattern	Oligodendrocyte origin Calcification + Fried egg cell appearance	Biopsy	Highest incidence is between 40 and 50 years of age. Most of the time, epileptic seizure is the presenting sign.
Meningioma	+	+/−	+/−	−	+	−	Well circumscribed Extra-axial mass Dural attachment CSF vascular cleft sign Sunburst appearance of the vessels	Arachnoid origin Psammoma bodies Whorled spindle cell pattern	Biopsy	Highest incidence is between 40 and 50 years of age. Most of the time, focal neurological deficit and epileptic seizure are the presenting signs. May be associated with NF-2
Hemangioblastoma	+	+/−	+/−	−	+	−	Infratentorial Cystic lesion with a solid enhancing mural nodule	Blood vessel origin Capillaries with thin walls	Biopsy	Might secret erythropoietin and cause polycythemia May be associated with von hippel-lindau syndrome
Pituitary adenoma ^[1]	−	−	+ Bitemporal hemianopia	−	−	Endocrine abnormalities as a result of functional adenomas or pressure effect of non-functional adenomas	Isointense to normal pituitary gland in T1	Endocrine cell hyperplasia	Biopsy	It is associated with MEN1 disease. Initialy presents with upper bitemporal quadrantanopsia followed by bitemporal hemianopsia (pressure on optic chiasma from below)
Schwannoma	−	−	−	−	+	−	Split-fat sign Fascicular sign Often have areas of hemosiderin	Schwann cell origin S100+	Biopsy	It causes hearing loss and tinnitus May be associated with NF-2 (bilateral schwannomas)
Primary CNS lymphoma	+	+/−	+/−	−	+	−	Usually deep in the white matter Single mass with ring enhancement	B cell origin Similar to non hodgkin lymphoma (diffuse large B cell)	Biopsy	Usually in young immunocompromised patients (HIV) or old immunocompetent person.
Childhood primary brain tumors
Pilocytic astrocytoma	+	+/−	+/−	−	+	−	Infratentorial Solid and cystic component Mostly in posterior fossa Usually in cerebellar hemisphers and vermis	Glial cell origin Solid and cystic component GFAP +	Biopsy	Most of the time, cerebellar dysfunction is the presenting signs.
Medulloblastoma	+	+/−	+/−	−	+	−	Infratentorial Mostly in cerebellum Non communicating hydrocephalus	Neuroectoderm origin Homer wright rosettes	Biopsy	Drop metastasis (metastasis through CSF)
Ependymoma ^[1]	+	+/−	+/−	−	+	−	Infratentorial Usually found in 4th ventricle Mixed cystic/solid lesion Hydrocephalus	Ependymal cell origin Perivascular pseudorosette	Biopsy	Causes an unusually persistent, continuous headache in children.
Craniopharyngioma ^[1]	+	+/−	+ Bitemporal hemianopia	−	+	Hypopituitarism as a result of pressure effect on pituitary gland	Calcification Lobulated contour Motor-oil like fluid within tumor	Ectodermal origin (Rathkes pouch) Calcification +	Biopsy	Initialy presents with lower bitemporal quadrantanopsia followed by bitemporal hemianopsia (pressure on optic chiasma from above)
Pinealoma	+	+/−	+/−	−	+ vertical gaze palsy	B-hCG rise leads to precocious puberty in males	Hydrocephalus (compression of cerebral aqueduct)	Similar to testicular seminoma	Biopsy	May cause prinaud syndrome (vertical gaze palsy, pupillary light-near dissociation, lid retraction and convergence-retraction nystagmus
Vascular
AV malformation ^[1]	+	+	+/−	−	+/−	−	Supratentorial: ~85% Flow voids on T2 weighted images	We do not perform biopsy for AVM	Angiography	We may see bag of worms appearance in CT angiography
Brain aneurysm	+	+/−	+/−	−	+/−	−	In magnetic resonance angiography, we may see aneurysm mostly in anterior circulation (~85%)	We do not perform biopsy for brain aneurysm	MRA and CTA	It is associated with autosomal dominant polycystic kidney disease, Ehlers-Danlos syndrome, pseudoxanthoma elasticum and Bicuspid aortic valve (Angiography is reserved for patients who have negative MRA and CTA)
Infectious
Bacterial brain abscess	+	+/−	+/−	+	+	Leukocytosis Elevated ESR Blood culture may be positive for underlying organism	Central hypodense signal and surrounding ring-enhancement in T1 Central hyperintense area surrounded by a well-defined hypointense capsule with surrounding edema in T2	We do not perform biopsy for brain abscess	History/ imaging	The most common causes of brain abscess are Streptococcus and Staphylococcus.
Tuberculosis ^[1]	+	+/−	+/−	+	+	Positive acid-fast bacilli (AFB) smear in CSF specimen Positive CSF nucleic acid amplification testing Hyponatremia (inappropriate secretion of antidiuretic hormone) Mild anemia Leukocytosis	Hydrocephalus combined with marked basilar meningeal enhancement	We do not perform biopsy for brain tuberculosis	Lab data/ Imaging	It is associated with HIV infection
Toxoplasmosis	+	+/−	+/−	−	+	Normal CSF	Multifocal masses with ring enhancement Mostly in basal ganglia, thalami, and corticomedullary junction.	We do not perform biopsy for brain toxoplasmosis	History/ imaging	It is associated with HIV infection
Hydatid cyst ^[1]	+	+/−	+/−	+/−	+	Positive serology (Antibody detection for E. granulosus)	Honeycomb appearance Necrotic area	We do not perform biopsy for hydatid cysts	Imaging	Brain, eye, and splenic cysts may not produce detectable amount of antibodies
CNS cryptococcosis	+	+/−	+/−	+	+	Positive CSF antigen testing (coccidioidomycosis) CSF lymphocytic pleocytosis Elevated CSF proteins and lactate Low CSF glucose	Dilated perivascular spaces Basal ganglia pseudocysts Soap bubble brain lesions (cryptococcus neoformans)	We may see numerous acutely branching septate hyphae	Lab data/ Imaging	It is the most common brain fungal infection It is associated with HIV, immunosuppressive therapies, and organ transplants In may happen in immunocompetent patients undergoing invasive procedures ( neurosurgery) or exposed to contaminated devices or drugs Since brain biopsies are highly invasive and may may cause neurological deficits, we diagnose CNS fungal infections based on laboratory and imaging findings
CNS aspergillosis	+	+/−	+/−	+	+	Positive galactomannan antigen testing (aspergillosis) CSF lymphocytic pleocytosis Elevated CSF proteins and lactate Low CSF glucose	Multiple abscesses Ring enhancement Peripheral low signal intensity on T2	We may see numerous acutely branching septate hyphae	Lab data/ Imaging	It is associated with HIV, immunosuppressive therapies, and organ transplants In may happen in immunocompetent patients undergoing invasive procedures ( neurosurgery) or exposed to contaminated devices or drugs Since brain biopsies are highly invasive and may may cause neurological deficits, we diagnose CNS fungal infections based on laboratory and imaging findings
Other
Brain metastasis ^[1]	+	+/−	+/−	+	+	−	Multiple lesions Vasogenic edema	Based on the primary cancer type we may have different immunohistopathology findings.	History/ imaging	Most common primary tumors that metastasis to brain: Lung cancer Renal cell carcinoma Breast cancer Melanoma Gastrointestinal tract If there is any uncertainty about etiology, biopsy should be performed

ABBREVIATIONS

CNS=Central nervous system, AV=Arteriovenous, CSF=Cerebrospinal fluid, NF-2=Neurofibromatosis type 2, MEN-1=Multiple endocrine neoplasia, GFAP=Glial fibrillary acidic protein, HIV=Human immunodeficiency virus, BhCG=Human chorionic gonadotropin, ESR=Erythrocyte sedimentation rate, AFB=Acid fast bacilli, MRA=Magnetic resonance angiography, CTA=CT angiography

References

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↑ ^1.0 ^1.1 ^1.2 ^1.3 ^1.4 ^1.5 ^1.6 Invalid <ref> tag; no text was provided for refs named :0

[nlmnihgov-1] National Library of Medicine.http://www.nlm.nih.gov/medlineplus/cancer.html

[2] Nicolato A, Gerosa MA, Fina P, Iuzzolino P, Giorgiutti F, Bricolo A. Prognostic factors in low-grade supratentorial astrocytomas: a uni-multivariate statistical analysis in 76 surgically treated adult patients. Surg Neurol 1995;44:208-21; discussion 221-3. PMID 8545771.

[3] Janny P, Cure H, Mohr M, Heldt N, Kwiatkowski F, Lemaire JJ, Plagne R, Rozan R. Low grade supratentorial astrocytomas. Management and prognostic factors. Cancer 1994;73:1937-45. PMID 8137221.

[4] Piepmeier J, Christopher S, Spencer D, Byrne T, Kim J, Knisel JP, Lacy J, Tsukerman L, Makuch R. Variations in the natural history and survival of patients with supratentorial low-grade astrocytomas. Neurosurgery 1996;38:872-8; discussion 878-9. PMID 8727811.

[nlmnihgov-1] 1.0 ^1.1 National Library of Medicine.http://www.nlm.nih.gov/medlineplus/cancer.html

[:0-1] 1.0 ^1.1 ^1.2 ^1.3 ^1.4 ^1.5 ^1.6 Invalid <ref> tag; no text was provided for refs named :0

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Brain tumor

Overview

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating brain tumors from other diseases

Epidemiology and Demographics

Risk factors

Natural History, Complications and Prognosis

Diagnosis

Staging and Grading

History and Symptoms

Physical examination

CT

MRI

Other imaging findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

References

Classification

Overview

Classification

Primary tumors

Secondary tumors and non-tumor lesions

References

Causes

Overview

Cause

References

Differentiating Brain Tumor from other Diseases

References

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