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Tumors of the CNS

Sean Hopkins

Professional Practice Leader- Neurosurgery

Hotel Dieu Grace Hospital

Tumors of Brain and Spinal Cord.

The objectives for today are:

• Identify the pathophysiology and

manifestations related to CNS tumors.

• Identify treatment options and nursing

strategies for a patient with a CNS tumor.

• Identify complications associated with CNS

tumors.

Statistics• Central Bran Tumor Registry of the United States

(CBTRUS) in 2007 reported > 51, 000 new cases of primary/secondary brain tumors. *

• Greater number of primary tumors in females*

• 12-13,000 deaths related to primary tumors*

• 2nd leading cause of death in males 20-39 years old in 1998,1999,2001. Highest cause death in 2000.*

• >30% are Supratentorial tumors*; Gliomas make 40% of those diagnosed tumors i.e. astrocytoma.

• GBMs making 25% Primary tumors.**

• It is estimated that 55,000 Canadians are surviving with a brain tumor**

*Hickey,6th edition, 2009.

** Brain Tumor Foundation Of Canada

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Causes Of Brain Tumors

• The causes of CNS tumors remains largely unknown.

• Possibilities include:

1. Genetics: a genetic predisposition such as

neurofibromatosis types ½, von Hippell-Lindau disease

or associated chromosomal link associated with

chromosomal locations and tumor formation.

2. Congenital factors associated with embryo growth

and development prior to birth.

3. Environmental agents such as ionizing radiation from

cosmic rays, electromagnetic , alpha, beta and gamma

rays, x-rays, radiation therapy.

Classification of Brain Tumors

• Tumor Node Metastases staging system is the

overall staging system; not clinically useful.

• Primarily brain tumors are classified by tissue of

origin and/or location within the brain.

• WHO ( World Health Organization) classification

grades tumors on a continuum into 9 categories

with subcategories of the major types.

• Conventional classifications include such factors

as: Primary vs. Metastatic, Neuroembryonic

origins, Anatomical Location and Malignant vs.

Benign

Conventional Classifications• Primary vs. Metastatic Primary brain tumors

originate from the various cells and structures

that make up the central nervous system.

Metastatic brain tumors originate from areas

outside the central nervous system. Common are

cancers of the lungs, breast.

en.wikipedia.org www.brainoncologycenter.com

VS.

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Conventional Classifications• Neuroembryonic origins

• Tumors that form from the ectodermic layer of the embryo during development. These glial cells are the basis of the broader category of CNS tumors called Gliomas:

• Astrocytomas

• Oligodendrogliomas

• Ependyomas en.wikipedia.org

Conventional ClassificationsAnatomic Location

Usually refers to Supratentorial, above

the tentorium cerebelli involving

the cerebral hemispheres or

Infratentorial or below the

tentorium cerebelli involving the

cerebellum and/or brainstem.

Location is important in deciding

treatment options, prognosis and

deficits associated with the

functional aspects to the location in

the brain. www.williamdoolin.com

Conventional Classifications• Malignant vs. Benign Difference is

made based on the histology

results. Malignant usually

associated with aggressive tumors

of poor prognosis.

• Benign suggests slower growing,

operable and curable. Benign

could be referred to as surgically

inaccessible leaving chemotherapy

and radiation as the treatment

options. However if these prove

ineffective, the tumor may still

grow and lead to neurologic

decline. Benign does not always

carry the cure factor.

www.masslive.com

High Growth= High Grade Tumor

Slow Growth = Low Grade Tumors

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Pathophysiology of Brain Tumors

• Brain Tumors grow from genetic mutations of brain matter cells that is not completely understood. This mutational change causes the cells to function abnormally, to grow inappropriately, and invade surrounding cells and brain tissue.

• As they grow they begin to affect the brain by compression, invasion and sometimes through erosion.

• Brian tumors can grow until forced to change direction because of bone or space i.e. falx cerebri

• Brain Tumors can grow diffusely without forming a definite mass therefore allowing multiple calls and cell growth, necrosis, fluid accumulation, bleeding and cell death.

Pathophysiology continued• Understanding the clinical effects of a brain tumor requires an understanding of

the Monroe Kelly Hypothesis.

• MKH- a balance between brain, blood and CSF within the cranial vault, changes

in one causes changes in the other 2 in an effort to compensate for the

disruption. Once these compensatory mechanisms have reached the limit-

deterioration progresses quickly. Focal deficits, changes are noted as the tumor

grows, sometimes missed or subtle until major event, such as seizure to bring it

into the light that changes were happening all along.

• Cellular Edema+ mass effect- pushing or shift causes the compensation of brain,

CSF and blood flow in an attempt to maintain perfusion and function. As the

mass effect and edema increases, the signs of increasing ICP become

increasingly visible. These continue as the tumor grows.

• Cerebral herniation and death are potential scenarios.

Staging of Brain Tumors

• Brain and Spinal Cord Tumors have no formal

staging system since they rarely grow or spread

outside of the Central Nervous System.

• Treatment can depend on factors such as:

-the type of the cell identified as the origin of the

tumor

-the location of the tumor in the brain or spinal cord.

-the amount of tumor remaining after surgery

-the grade of the tumor

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Signs and Symptoms

• Nothing clear and concise.

• Symptoms depend on the size, location,

compression of specific brain tissue , amount of

edema and if ICP is increased.

• 2 groups- General and Focal.

curehemorrhoidsrapid.com

General Signs and Symptoms

• Headache- most common

presenting symptom.

• Often worse in the

morning/awakens at night

• Aggravated by coughing,

straining, bending, lifting

• Generally progresses to

bifrontal or bioccipital

General Signs and Symptoms

• Nausea and Vomiting

Mainly indicate posterior fossa tumors and/or Increasing ICP

Not necessarily with a headache.

Vomiting can be unrelated to meals

Stimulation of vomiting center located in the Medulla region from stimulation/compression-more common with posterior fossa.

www.ilmiomondocinema.com

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General Signs and Symptoms

• Change in the Level Of Consciousness

Subtle changes may go unnoticed at first-slow growing tumors- slow progression weakness and confusion

Slight confusion, irritability, somnolence progressing to stupor, coma and death.

Rapid deterioration usually related to sudden increases ICP- if it bleeds.

AWAKE

COMA

L

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General Signs and Symptoms• Seizures

If this is NEW, then it needs to be investigated for a

cause!!!

• 30% it’s the initial reason for admission> discovery

of the tumor

• Seizures will commonly happen ~30% tumors

diagnosis: Rapid growing tumors have seizures 30-

40% while slow growing tumors ~30%

• Primarily in Supratentorial tumors- although focal

seizures may aid in localizing area of activity.

Focal Signs and SymptomsLocalized signs and symptoms specific to brain

region/lobe may show specific signs when

assessing the patient for/with a brain tumor:

Frontal Lobe- Anterior and Posterior regions

Parietal Lobe- Sensory and Recognition/Body Orientation

Temporal Lobe- Psychomotor

Occipital Lobe- Vision

Pituitary/Hypothalamus- metabolic and endocrine

Lateral/3rd Ventricles- CSF problems

Brainstem- motor and sensory

Midbrain

4th Ventricle-

Cerebellum- Balance and Coordination

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Frontal Lobe- aka Frontal Lobe Syndrome

Anterior or Posterior aspects can give some discerning characteristics:

Anterior Frontal Lobe

Higher Level Cognitive Personality

Short Term Memory Emotional Labile

Long Term Memory Flat affect

Concentration no Initiative

Slow Mental Processing and Loss of Self Restraint

Reaction time Loss of Social

Calculations and problem solving Behavior Control.

Abstract Thinking

Posterior Frontal Lobe:• Location within the

posterior frontal lobe might be the key.

• Brocas area- dominant hemisphere related tumor; expressive and fluent speech; word finding

• Motor weakness and possible seizure activity with location close to the motor strip in posterior aspect of frontal lobes.

www.cafemuse.com

Parietal Lobe: Sensory/Recognition/Orientation

Sensory Recognition Orientation-Tactile Disturbances -Difficulty recognizing letters -loss of Left-Right

and numbers discrimination

-Abnormal Sensations -Inability to recognize object -Difficulty going

(tingling, crawling, burning) size, shape, feel. Through

doorways

-Loss of 2 point -inability to recognize -neglect syndrome

discrimination parts of the body opposite to tumor

-denies sensory/motor loss - construction apraxia

( clock face test)

• Parietal Lobe Syndrome- collection of

various aspects and deficits from these

sub-categories.

www.memorylossonline.com

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Temporal Lobe• Seizures- Visual, auditory, motor with

amnesia of events. Aura/ “strange

sensation” in epigastrium or thorax.

• Weakness

• Visual field deficits

• Short term memory deficits- recent

events.

• Speech and Language problems-

dominant hemisphere/Wernicke’s

areawww.cafemuse.com

Occipital Lobe• Not as common when compared to other areas.

• Cause vision disturbances- loss of vision in visual fields or failure to

recognize familiar objects.

www.health.qld.gov.au

Pituitary & Hypothalamus Tumors• Related by location and endocrine

production

• Visual deficits- paralysis/optic atrophy/pressure on the optic/cranial nerves operating the eye(s)

• Pituitary dysfunctions – Cushing's syndrome, acromegaly, giantism

• Hypothalamus- water balance sleep pattern appetite, sexual drive disturbances

• Hormone production and release disturbances.

www.mayoclinic.com

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Lateral & 3rd Ventricles• Small tumors may be and remain

asymptomatic

• Growing tumors will cause problems in the

area of the brain involved.

• Commonly cause CSF obstruction leading to

hydrocephalus with signs of increasing ICP-

headache, nausea, vomiting

• Some report relief of position of the head

changes- moving the tumor and relieving the

obstruction to CSF flow.

www.netterimages.com

Midbrain Tumors• Midbrain tumors tend to

rare

• Affect the cerebral aqueducts and cerebellum

• Parinaud’s syndrome-paralysis of upward gaze

• Abnormal posturing

• Ptosis

• Handles reflexes of eye

movements

• Controls posture and

walking

brainavm.oci.utoronto.ca

columbia.edu

Brainstem Tumors• Lower Cranial Nerve deficits – swallowing -gag reflex

talking

• Motor/sensory deficits

• Vertigo

• Hiccups

• Ataxia

• Dizziness

• Coordination problems

• Obstructive Hydrocephalus from CSF flow disturbance

• Death from compression on vital centers- respiratory and cardiac.

www.isabellaandmarcusfund.org.au

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Cerebellum• Ataxia

• Incoordination

• Nystagmus

• Dizziness

• Nausea

• Signs of hydrocephalus

and increasing ICP from

CSF flow obstruction

• lethargic

www.roberthardwick.com

Diagnosis of a Brain Tumor

• Neuro-Imaging performed through 2 ways:

• Computed Tomography -CT Scan- with and without

contrast

– Works great if MRI cannot be done ( pacemaker etc…)

– Characteristics of metastatic disease can be seen

www.radiology-equipment.com

www.neurology.org

Diagnosis of a Brain Tumor

• Magnetic Resonance

Imaging> MRI with

gadolinium

– Highly sensitive to structures

– Identify structural

abnormality and edema

– multiplane images > accurate

localization and identification

of tumors without losing

detail

www.dna2life.com

www.fmrib.ox.ac.uk

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Diagnosis of a Brain tumor.• Endocrine laboratory studies for pituitary tumors

may have significance to location and

involvement of anterior or posterior divisions of

the pituitary gland

• Acoustic tumors may use some degree of

audiometric studies to determine the degree of

hearing loss

Pharmacology• Corticosteroids

• Dexamethasone ( Decadron); Prednisone

WHY: Used to decrease Cerebral EdemaMay be started immediately ,after diagnosis is confirmed, or edema and signs of increased

ICP are present .

Causes:

• GI issues and peptic ulcer disease- should be given with a GI prophylaxis medications to decrease the risk of developing a peptic ulcer. Medication to prevent PUD.

• blood glucose fluctuations

• fluid retention

• Cushing’s syndrome

• Decreased wound healing

• Psychosis/Delirium

• Patients own medication reconciliation something to consider.

Pharmacology

Seizure prophylaxis• Controversial “No study to date supporting their use” ( Hickey, 2009;p511.)

• Physician preference to what agent to use. Common is Dilantin ( Phenytoin), but this not

without its issues, especially IV.( hypotension, PIV vasculitis, tissue damage if

extravagated, ISMP Canada Alert February 2013)

• Common 1 gram bolus dose then 100 TID or 300 once daily- dictated by drug levels to

achieve the therapeutic levels to control seizures.

• Oral form is absorbed well gastric, unless there are gastric issues and absorption issues

due to lack of appetite, not eating because of the brain tumor. Oral liquid has

restrictions with tube feedings, altering nutrition and feeding rates and calorie intake.

• Other agents may include: Tegretol, Keppra,

• Goal is to prevent seizures from happening due to the effects

they have on brain oxygenation, brain tissue survival if already

compensated to mass effect of an invading tumor.

• More common Supratentorial lesions- similar to TBI where sensory/motor strip can be

reached and spread small seizure to full tonic clonic seizure activity.

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Surgery• GOAL- safe removal of as much visible tumor as possible without causing neurological deficit.

( Hickey, 2009)

• Not always possible depending on tumor location and infiltration of vital areas i.e. brainstem

• Possible “ cure” for benign tumors where removal of the entire tumor is achieved with no further

therapy.

• Partial Tumor Resections can be achieved for those tumors that cannot be totally removed- MRI and

digital scans assist in this boundary mapping to maximize removal and resection along with

embolization prior to surgery to decrease bleeding for certain tumors.

• Biopsy- open or stereotactic obtains samples but does not decrease the tumor or its effects on the

brain.

• Craniotomy with removal of bone flap- resection/removal of the tumor – replace the bone flap.

Radiation Therapy2nd option in treating brain tumors in combination with surgery.

GOAL: To target the residual tumor that remains in hopes of reducing

or stabilizing its size to prevent or delay tumor re-growth.

Surgical resection > microscopic seeding in surrounding tissue>

tumor re-growth or metastasize.

Post-operative radiation therapy can be used in cases of benign or

malignant tumors.

HOW: Tumor cells are more susceptible to radiation due to their

increased mitotic activity. By damaging the DNA of the dividing

tumor cells, the growth process is halted, or at least delayed.

Radiation Therapy• Types include:

1. EBRT- External Beam Radiation Therapy

-Conventional for inoperable or sensitive tumors.

-Uses X-ray radiation

- Focal EBRT radiates the tumor only

- Whole Brain XRT radiates the entire brain.

2. Proton Therapy uses proton particles and is 3 dimensional in its

approach to radiating the tumor using a computer.

3. Conformal Radiation conforms the radiation to shape match the

tumor, minimizing exposure to surrounding tissue. Radiosurgery

with the GAMMA Knife uses this type of approach.

4.Interstial Brachytherapy involves surgically implants radioactive

SEEDS into the tumor bed; Temporary or permanent.

en.wikipedia.org

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Radiation Therapy

• Not always successful- tumor grows back.

• Rounds of same surgery/radiation may be

needed.

• Side effects

• Somnolence, hair loss, skin disorders.

• Decreased cognitive functions, hydrocephalus,

cerebral necrosis.

Chemotherapy• 3rd Component of the multimodal approach that utilizes Surgery

and Radiation.

• Can be given before, during or after radiation therapy

• Reoccurrence is primary use.

• Oral or IV administration is the most common.

• Interstitial administration- Impregnated wafers directly on the

tumor bed

• Intrathecal- delivers chemo drugs right into the spinal fluid.

• Intra-Arterial route as opposed to IV route.

• Preference is determined by MD and best course to

attack the tumor to get the best results.

Chemotherapy• Cytotoxic- agents that cause CELL DEATH

• Cytostatic- agents that cause cells to behave normally.

• NOT always successful! Blood Brain Barrier often blocks the effectiveness of the agents to get to the tumor, or the cells become resistant to the chemo-agents.

• SIDE EFFECTS:anemia, leucopenia, nausea, vomiting, fatigue etc…

• Cerebral edema can hinder the drugs effectiveness

• Drug resistance can develop which can lead to different agents being used in reoccurrence.

• Chemotherapeutic Drugs Commonly Used to Treat Brain Tumors

Joann V Hickey, Clinical Practice of Neurological and Neurosurgical Nursing,2009, 6th Edition, pg.513.

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Meningioma

• Commonly originates in Dura or Arachnoid mater of the brain and or spinal cord.

• Compresses rather than invades cerebral or neural structures

• Seen on CT scan/enhanced CT scan

• Tumor site and size influences the symptoms seen. May be slow progression with slowly evolving symptoms, weakness, speech, comprehension, confusion, balance, vision, if seizures presenting symptom.

• Surgically resectable by craniotomy is possible however reoccurrence is probable if total resection is incomplete.

• Radiation often with incomplete resection to decrease reoccurrence chances.

Meningioma• Complete neurological exam can

detect weaknesses or changes from the baseline including sensory and motor evaluations.

• Type of Surgery and date performed is important for post-op complications and evaluating neurological changes.

• Surgical Site examination for infection/bleeding/leaking CSF

• Pre/post –op pain control for headaches

• PT and OT for evaluation of any deficits and weakness with ADLs To evaluate and assist for deficits from motor involvement.

Glioblastoma Multiform • One of most common brain neoplasm

• Originates in the brain matter; less common brainstem or in children

• Primary or secondary( Mets) formation

• Presentation history usually headaches first, commonly Morning

headaches

• Progressing motor weakness, seizures, nausea, vomiting, cognitive

impairment, changes to personality, mood, attention, concentration,

mental capacity.

• Symptoms often dependent on section of brain the lesion develops.

• Sensory and motor loss, hemi paresis developing and worsening with

time.

• Surgery/chemotherapy/radiation often goals for therapy.

• CT result identifying the location of the lesion.

• This often will tell you the deficits that

may be seen based on the area of the brain

and extent of the size of the tumor.

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Complete neurological exam including cranial

nerves can determine deeper CNS involvement

that may impact things like vision, movement,

paralysis, speech and swallowing, cognitive

functions

Observe surgical site for infection, leaking,

bleeding.

Post-op pain control medications needed.

Cognitive deficits may pose a fall risk or need

for restraints for patient safety.

Steroids may be ordered for edema then

tapered off or discontinued

Seizure control medications

PT and OT required for assistance with

ambulation and ADLs plans.

Palliative treatment may be in the end goal.

Often reoccurs in/close to original tumor site.

Surgical Intervention:

Crainiotomy and removal of the tumor.

Radiation

Chemotherapy:

Gliadel wafer implants for higher

staged tumors-pose risks-seizures,

brain edema, mass effect.

Nursing Considerations

en.wikipedia.org

Astrocytoma

• Slow growing Primary tumor that originates in

astrocyte cells of brain matter:

• Symptoms arise from pressure exerted inside

the skull on brain matter;

• Symptoms are dependant on tumor location as

it develops and size upon discovery.

• Headache, nausea, vomiting from increased

ICP.

• Drowsiness and lethargy

• Mental status changes including but not limited

to cognition, personality, disorganized thinking,

concentration.

• Diagnosed on CT/enhanced CT/ MRI

• Surgical intervention with removal of tumor

often possible but not always successful in

removal of all the tumor.

emedicine.medscape.com

Astrocytoma• Location and extent of the tumor/surgery to determine

the expected and potential neurological effects based on brain location, lobe and functions affected by the brain tumor( i.e. Sensory and motor, speech)

• Neurological exam with baseline history is important to detect and evaluate deficits and changes prior to surgery and changes after surgery.

• Surgical site evaluation for bleeding and infection. Drains might be present.

• Pain control medications.

• Seizure control medications

• PT and OT to evaluate ADLs and mobility needs/deficits post-op.

• Speech and Swallow study to evaluate eating/swallowing issues.

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Pituitary Tumors• Pituitary Gland rests in the sella turnica of the sphenoid bone

• Pituitary gland regulates the function of the thyroid & adrenal glands , the kidneys, plus the ovaries and testes as well as regulates growth

• Two parts: Anterior=adenohypophesis

( ACTH, TSH, FSH, LH,GH,PRL )

Posterior=neurohypophesis ( ADH, oxytocin)

ADH is the hormone responsible for water reabsorbsion by the kidneys

• Controls various biochemical and physiological functions by secreting hormones: 2 posterior and 6 anterior.

• Dysfunctions are affected by location of tumor in pituitary and affect the functions associated with those hormones i.e. Diabetes Insipidus

• Surgical removal involves

removal of the entire pituitary gland

-transsphenoidalhypophosectomy.

Transphenoidal Surgery• Surgical incision assessment. May be in the mouth under

the upper lip.• Evaluation for potential CSF leaks- continuous nasal drip/

salty taste mouth.• Excessive urination/voiding/dumping dilute urine• Excessive thirst• Vital signs- may become hypotensive and tachycardia if

hypovolemia develops.• Lab values including urine and serum electrolytes, serum

osmolality elevation, urine specific gravity decrease elevation serum sodium.

• Medications may include hormone replacements and Aqueous vasopressin (DDAVP) in various forms i.e. nasal spray, s/q injection.

Also see Care if the Patent with Diabetes Insipidus presentation for specific care for DI

•Cerebellar-Pontine Angle Tumors

•Primary Central Nervous system

Lymphoma

•Medulloblastoma

•Ependymoma

Refer to Hickey, 2009 pages 502 to 506, Table 21-1

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Spinal Cord Tumors

• Tumors compress the spinal cord- externally or on the cord itself.

• Most common are metastatic tumors that cause external

compression. i.e. breast, lung, prostate, colon, uterus, kidneys

• Classified by:

1. Relationship to the Dura and Spinal Column

Extramedullary-

outside spinal column> Extradural-outside the Dura

> Intradural- within the spinal Dura

Intramedullary- within the substance of the spinal cord; usually

primary spinal cord tumors

2. Location in relation to the Vertebral Column

Spinal Cord Tumors.Signs and Symptoms will depend on aspects of:

• level of the tumor on the spinal cord called “ Spinal Level”

• Compression of the spinal cord

• Ischemic changes secondary to arterial or venous

obstruction of the spinal cord

• Direct Invasion of the cord

• PAIN Evolving over time period; localizing and point specific/tender

with developing reticular pain experienced along the nerve roots and

the dermatomes being affected.

• Pain is exaggerated by movement, coughing, reclining, straining ranging

form dull to piercing/stabbing/burning

Spinal Cord Tumors

• Motor / Sensory deficits below the level of the lesion producing motor weakness, wasting, hypotonic, loss of reflexes, paralysis

• Bowel ,Bladder and Sexual Dysfunction- paralytic ileus

and constipation/ urgency progressing to retention

and impotence.

• Froin’s Syndrome- CSF locked below the level of the tumor causes the CSF to clot immediately, contain high protein counts with few or no cells.

Similar to Brain tumors, subtle signs may go unnoticed until detailed history of symptoms reveals a long evolving pattern involving the key clinical symptoms of pain, motor and sensory deficits, bowel, bladder or sexual dysfunction.

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Spinal Cord TumorsDiagnosis: CT scan and MRI scan to define

and evaluate the extent of the spinal

cord involvement.

MRI with contrast can provide detection

within the bony areas such as vertebra

bodies as well as enhanced mutiaxial

views to provide better tumor detection,

delineation, and grading.( Hickey, 2009)

www.columbianeurosurgery.org

Spinal Cord Tumors• Treatment of a Spinal Cord Tumor focuses

on certain goals of therapy that include:

1. Preservation of Neurological Function

2. Control of the PAIN

3. Plan to Remove/Control the Tumor.

Corticosteroids Prevent the irreversible

infarction/damage to the spinal cord

• Combination of Surgery, Radiation and

Chemotherapy are determined by the

location of the tumor, type of tumor an

the rate at which it is growing to cause

worsening developing symptoms.www.onlinecancerguide.com

Complications of CNS Tumors

• Seizures- symptom- prophylactic treatment.

• Fluid /electrolyte imbalance

• Hydrocephalus- secondary/obstructive

• Edema- Brain or Spinal Cord

• Cranial Nerve Deficit/ Palsy-location?

• Pain! Pain! Pain!

• Neurogenic bowel and bladder- loss of normal function.

• CSF leak- may heal on its own; might not.> Surgery?

• Sensory motor dysfunctions

• Syrinx- aka pseudo cyst or fluid filled cavity the surrounds a tumor that may require draining.

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References

• Joann V. Hickey, The Clinical Practice of Neurological

and Neurosurgical Nursing( 2009). 6th Edition.

• Journal of Neurosurgery, http//thejns.org

• The National Cancer Institute, www.cancer.gov

• The Brain Tumor Foundation Of Canada.

• Brain Tumor Foundation of Canada,

www.braintumour.ca

• Brain Tumor Foundation,

www.briantumorfoundation.org

• American Association of Critical-Care Nurses