Brain tumors - Lippincott Williams & Wilkins...United States, brain tumors are the deadliest of childhood cancers. And just because a brain tumor is benign doesn’t mean it’s harmless;

Primary malignant brain tumors in adults exist in a dizzyingarray of forms. Find out about the types of tumors, theirsigns and symptoms, treatment options, and effectivepatient management.DAWN CAMP-SORRELL, FNP, AOCN, MSNOncology Nurse Practitioner • Hematology and Oncology Associates • Sylacauga, Ala.

The author has disclosed that she has no significant relationships with or financial interest in any commercial companies that pertain to this educational activity.

ONE OF THE most chilling pronouncements anyone canhear is, “You have a brain tumor.” The fear this declarationcauses the patient is often justified, especially when thetumor’s malignant, invasive, fast-growing, and life-threat-ening. Existing treatments can be ineffective.

Tumors that originate in the brain vary widely by tissuetype, site of occurrence, growth potential, tendency toprogress, tendency to recur, and treatment response.According to the Pediatric Brain Tumor Foundation of theUnited States, brain tumors are the deadliest of childhoodcancers. And just because a brain tumor is benign doesn’tmean it’s harmless; a benign tumor in the wrong place canbe deadly. Regardless of their characteristics and point oforigin, however, malignant brain tumors have the highest

morbidity and mortalityrates.

Tumors originatingfrom structures withinthe brain are called pri-mary brain tumors.Those that spread fromother areas in the bodyto the brain are calledsecondary, or metastatic,brain tumors. In this

20 Nursing made Incredibly Easy! May/June 2006

Brain tumorsFacing trouble head-on

By the numbersPrimary brain tumors represent 1.4% of allcancers diagnosed in the United States.About 18,500 primary malignant brain tumorsare diagnosed each year. If you include all pri-mary brain tumors, the number rises toaround 41,000 new cases each year. Almost13,000 deaths annually are caused by braintumors.

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No easy wayaround thisdiagnosis,that’s for

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May/June 2006 Nursing made Incredibly Easy! 21

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article, I’ll focus principally on primarymalignant tumors in adults. I’ll discusscauses, pathophysiology, clinical presenta-tion, diagnosis, and treatment options, aswell as go over some important new treat-ment options.

First, let’s review the brain’s anatomy.

Central processingunitThe brain consists of soft,spongy tissue divided intothree major structures: thecerebrum, the cerebellum, andthe brain stem. It’s coveredand protected by the skin, theskull, and the meninges. Cere-

brospinal fluid bathes the tissueand cushions it from shocks.The outer layer of the cerebrum, the

cerebral cortex, consists of unmyelinatednerve fibers called gray matter. Within thecerebrum are myelinated nerve cells calledwhite matter. Basal ganglia, which controlmotor coordination and steadiness, are

found in the white matter.The undulating surface of the cerebrum is

made up of gyri (convolutions) and sulci(creases). The cerebrum has a right and lefthemisphere. The corpus callosum, a mass ofnerve fibers, bridges the hemispheres, allow-ing communication between correspondingcenters on each side. Some of the large sulcidivide the surface into lobes: the frontal, theleft and right parietal, the left and right tem-poral, and the occipital. The lobes are namedafter the cranial bones that cover them.

The cerebellum is underneath the backpart of the cerebrum. It also has two hemi-spheres, each having an outer cortex ofgray matter and an inner core of white mat-ter. This part of the brain maintains muscletone, coordinates muscle movement, andcontrols balance.

The brain stem lies just below the cere-brum, in front of the cerebellum. It runs fromthe cerebrum and connects to the spinalcord, and it consists of the midbrain, thepons, and the medulla oblongata. Its func-tion is threefold: (1) to mediate autonomiccardiac, respiratory, and vasomotor function;(2) to provide two-way communication linesfor nerve signals; and (3) to serve as the ori-gin for 10 of the 12 pairs of cranial nerves.


Where the trouble startsPrimary brain tumors are classified accordingto histology (tissue of origin), as follows:• Tumors originating in neuroepithelial tissue

—astrocytomas—glioblastomas—oligodendrogliomas—mixed gliomas—ependymal tumors• Tumors originating in cranial and spinalnerves

—central nervous system lymphoma• Tumors of sellar region

—pituitary adenomas—pituitary carcinomas—craniopharyngiomas—chordomas, chondrosarcomas.

Let’s take acloser look at what’s

inside you.

Gyri

Central sulcus

Lateral sulcus

Parietal lobe

Occipitallobe

CerebellumSpinal cord

Medullaoblongata

Pons

Temporal lobe

Frontal lobe

Frontal lobe

Anatomy of the brain

The diencephalon is the part of the brainlocated between the cerebrum and the mid-brain. It consists of the thalamus, the hypo-thalamus, the subthalamus, and the epithala-mus, which lie deep in the cerebral hemi-spheres. All sensory information (exceptfor olfactory information) has to check in atthe thalamus before it can be transmittedappropriately to the brain. The hypothala-mus helps to power autonomic functions,like regulating body temperature, hunger,hormone output, and the sleep/wake cycle.

Those are the parts. Now let’s briefly lookmore closely at the types of cells that makeup the various brain structures.

The nervous system contains two types ofcells: neurons and glial cells. The neurons arethe basic anatomic and functional units ofthe nerves. The glial cells provide nourish-ment, structural support, and protection for

the neurons. Within the brain and spinalcord, glial cells surround nerve cells andhold them in place. They’re subdivided intofour main types: astrocytes, oligodendro-cytes, ependymal cells, and microglia.Astrocytes and oligodendrocytes are specifictypes of neuroglial cells (nonneural support-ing elements). Oligodendrocytes formmyelin in the central nervous system (CNS).

Now that we’ve got the architecture inplace, it’s time for a look at what can gowrong when a tumor arises in one or moreof these structures.

Invasion forceExperts haven’t yet found any positivecauses or risk factors for primary braintumors. In a few rare hereditary syn-dromes—NF1, NF2, Turcots, Gorlins,tuberous sclerosis, and Li-Fraumeni—


Making the gradeBecause the tumor-node-metastases (TNM) staging system used for many other cancers doesn’twork very well for brain tumors, classification is based on alternate systems. Following are two of themost widely used: the World Health Organization (WHO) classification of nervous system tumors andthe National Cancer Institute’s (NCI) grading system for adult brain tumors.

WHO grade Characteristics

I Discrete lesions with low proliferative potential. May be curable with surgical resection.

II Infiltrating lesions that are low in mitotic activity but recur. Some tumor types tend to progress to or recur at higher grades of malignancy.

III Lesions with histologic evidence of malignancy in the form of mitotic activity, clearly expressed infiltrative capabilities, and anaplasia.

IV Lesions that are mitotically active, necrosis-prone, and associated with a rapid preoperative and postoperative evolution of disease.

NCI grade Characteristics

I The tumor grows slowly, has cells that look similar to normal cells, and rarely spreads into nearby tissues. It may be possible to remove the entire tumor by surgery.

II The tumor grows slowly, but may spread into nearby tissue and may become a higher-grade tumor.

III The tumor grows quickly, is likely to spread into nearby tissue, and the tumor cells look very different from normal cells.

IV The tumor grows very aggressively, has cells that look very different from normal cells, and is difficult to treat successfully.

there’s a genetic link to brain tumor devel-opment. Links to environmental exposuresare under scrutiny; only one, exposure toionizing radiation, is known to increasethe risk of a primary brain tumor.

Primary brain tumors are classifiedaccording to the tissue of origin or histol-ogy—how it looks under a microscope (seeWhere the trouble starts). Two grading sys-tems are commonly used (see Making thegrade). The higher the grade, the moreaggressive the tumor will be. Tumors oftencontain several grades of cells: the highestgrade determines the grade by which thetumor is classified. Further classification isbased on genetic tumor markers and molec-ular biologic abnormalities.

The signs and symptoms of a brain tumordepend on its size, type, and location.

Tumors can arise from the brain tis-sue itself or from the meninges,

pituitary gland, or cranial bloodvessels (see A glossary of com-

mon tumor types). Symptoms,often characterized by insidious

onset, are typically caused by an

increase in intracranial pressure (ICP) andblockage of cerebrospinal fluid. Some of themost common signs and symptoms include:n frequent headachesn decreased motor strength and coordina-tionn seizuresn altered vital signsn vomiting with or without nausean papilledeman anorexian changes in mood/personalityn changes in the ability to think andlearn.

Most patients present with a headachethat won’t go away with pain medication.Headaches occurring on awakening can bean early symptom of brain tumor. They maybe associated with projectile vomiting, whichsuggests an increase in ICP. The headachesmay be dull or sharp and, as the ICP rises,tend to be frontal or occipital.

Some patients have changes in mentalstatus, mood, or personality. They may alsosuffer memory loss, lose the ability to con-centrate, or exhibit focal neurologic signs.


A glossary of common tumor types

Tumor type Description

Gliomas (account for Tumors of the neuroepithelial/glial cells40% to 50% of intracranial neoplasms)

• Astrocytomas • Overgrowth of astrocyte cells (the brain’s connective tissue)

• Oligodendrogliomas • Overgrowth of oligodendrocyte cells with calcification

• Ependymomas • Overgrowth of the ependymal cells

• Mixed gliomas • Two or more cell types within a tumor

• Medulloblastomas • Most common pediatric malignant tumor. Rapid growth pattern, highly invasive with a high risk of metastases

Meningiomas Arise from the linings (meninges) of the brain and can involve the skull

Peripheral nerve tumors Include acoustic neuroma, schwannoma, and Von Recklinghausen’s(type 1) neurofibromatosis and type 2 neurofibromatosis

Pituitary adenomas Can be secreting or nonsecreting

Germ cell tumors Dermoid cysts, epidermoid cysts, pineal tumors, and chordomas

Hematopoietic tumors Primary malignant lymphomas

I haven’tbeen

myselflately!

Delirium and dementia are often indicatorsof a brain tumor. These symptoms are poor-ly understood, and they can cause distressfor the patient and his family.

Brain tumors can produce irritation thatcauses seizures. Common types of seizuresthat arise from brain tumors include simplefocal, complex partial, and generalizedtonic-clonic. Simple focal seizures are local-ized; that is, they’re produced in a specificpart of the brain. These seizures involvesimple sensory or motor functions. Com-plex seizures occur when the brain irritationspreads into the frontal and temporal lobes.In a generalized seizure, the irritation isspread over both sides of the brain; thepatient may lose consciousness and hisarms and legs will move uncontrollably.Loss of bladder and/or bowel control mayoccur (see Brainstorm: Managing a seizure).

These signs and symptoms may point toa brain tumor, but they’re not definitive.Let’s look next at ways to diagnose a braintumor.

Picture thisDiagnosis begins with a thorough history.Ask the patient to describe his symptoms,calculate how long he’s had them, whenthey occur, whether he sees a connectionto an activity or circumstance, and if theyseem to be worsening.

A neurologic exam is the next order ofbusiness. This involves various tests ofmovement, vision, touch, hearing, smell,reflexes, eye response, balance and coordina-tion, abstract thinking, mental status, andmemory. Abnormal findings may be able tohelp pinpoint the area of the brain that’saffected.

Computed tomography (CT) scan is themost widely used diagnostic imaging test.Magnetic resonance imaging (MRI) is alsoused; it’s more sensitive for detecting intra-parenchymal brain tumors like ependymo-mas, the most common brain tumor in chil-dren. Hemodynamic imaging combines CTscan or MRI with the ability to gauge the

speed of contrast dye uptake; it’s useful forshowing tumor neovascularization.

Cytologic studies of cerebrospinal fluidcan be used to detect the presence of malig-nant cells shed by the tumor. An electroen-cephalogram can detect areas where abnor-mal brain waves occur and evaluate tempo-ral lobe seizures. It can also be used to helprule out other disorders.

Once a lesion is pinpointed, a neurosur-geon will obtain a biopsy specimen via cra-niotomy to determine the histology or the tis-sue type of the tumor. Stereotactic (computer-directed) biopsy is an option when the lesionis too deep or in an area where craniotomy istoo risky or when the patient can’t tolerategeneral anesthesia.

Next up: treatments.

Reducing planWhenever feasible, treatment for a braintumor begins with surgery. The primarygoal is to excise as much of the tumor aspossible to reduce the severity of signsand symptoms while optimally preservingneurologic function (see Craniotomy: A holein the head for postsurgical management).Let’s look at the other options that goalong with surgery.


Craniotomy: A hole in the headAfter a craniotomy, the patient is closely monitored in the intensive careunit. Be particularly aware of potential complications, like intracranialbleeding, cerebral edema, and water intoxication. A thorough neurologicassessment can detect the patient’s level of consciousness, intracranialpressure (ICP), and motor dysfunction. Decreased strength or level of con-sciousness and pupil changes may indicate increased ICP and cerebraledema.

• Monitor vital signs. Increasing temperature may indicate infection;increased heart rate or blood pressure may indicate increased ICP.

• Discourage activities that may increase ICP, such as straining.

• Monitor for seizure activity.

• Assess for signs of intracranial bleeding, such as swelling over the inci-sion, changes in mental status, nausea, or vomiting.

• Provide support to the patient and family during the immediate postop-erative period.

n Radiation. Radiation therapy is usefulfor improving cure rates and prolongingsurvival for most types of primary braintumors. The type of radiation technique

used and the amount given de-pends on tumor histology, grade,and location and the patient’sphysical status. Ionizing radia-tion targeting the tumor is deliv-

ered so as to minimize injuryto the surroundinghealthy tissue. The most

common technique usedis external beam radia-tion therapy. As the

name implies, the radia-tion comes from a machine

outside of the body. Treat-ment is generally given 5

days a week for 4 to 6 weeks for a totaldose ranging from 40 to 70 Gy. Adverseeffects may include hair loss, fatigue, nau-sea, vomiting, scalp dermatitis, andheadache. Somnolence syndrome, charac-terized by mild drowsiness and fatigue tomarked lethargy, is common after brain ir-radiation. It usually subsides 2 to 3 weeksafter the start of therapy.

Stereotactic radiosurgery allows deliveryof high-dose radiation in a single dose to a

precise area in the brain. The goal of thistechnique is to eradicate the tumor cellswithin a targeted area and minimize dam-age to the surrounding tissues. Adverseeffects may include headache. Also, theframe used to immobilize the patient’shead during this procedure may be uncom-fortable.n Chemotherapy. Chemotherapy has beenshown to boost survival in patients withgliomas, medulloblastomas, and certainother tumors. How well it works dependson the ability of the chemotherapeuticagent to cross the blood-brain barrier, aprotective mechanism the body uses tostop potentially damaging substances frominfiltrating the CNS. A few brain tumorsactually serve to break down the barrier.Chemotherapy is generally used as adju-vant therapy to surgery and radiation. Itcan also be used for recurrent brain tumors. n Drug therapies. The combination of a corticosteroid like dexamethasone(Decadron), an osmotic diuretic like man-nitol, and a loop diuretic like furosemide(Lasix) is used to reduce edema aroundthe tumor. Otherwise, the swelling couldcause brain structures to shift and increaseICP. The osmotic diuretic pulls fluid outof the space surrounding the tumor and


Match the treatment to the tumor type

Tumor type Radiation/surgery Chemotherapy

Lymphomas Whole-brain X-ray therapy Intrathecal ethotrexate, procarbazine, vincristine, high-dose cytarabine

Medulloblastomas Surgery, craniospinal radiation Lomustine, cyclophosphamide, vincristine, cisplatin, etoposide

Gliomas Surgery, radiation Temozolomide, carmustine, procarbazine, carboplatin, irinotecan, etoposide

Astrocytomas Surgery, radiation Temozolomide, carmustine, procarbazine, carboplatin, irinotecan, etoposide

Oligodendrogliomas Surgery, radiation Procarbazine, lomustine, vincristine

Meningiomas Surgery, external beam or Hydroxyurea, alpha interferon stereotactic radiation

With braintumors,

smaller isbetter.

into the intravascular space, where theloop diuretic can push the kidney to ex-crete it. A steroid can also help to relieveheadaches by lowering the ICP. Patientstaking a steroid will need ulcer prophy-laxis with a histamine2-receptor blockerlike famotidine (Pepcid) or a proton pumpinhibitor like lansoprazole (Prevacid).Seizure medication may also be used. As-pirin and anticoagulation medications arestopped to avoid intracranial bleeds.

Medications can be used in the nonspecifictreatment of concentration and memory dis-turbances to manage coexisting depression,anxiety, or agitation.

See Match the treatment to the tumor typeand Plan B for more information.

Handling the tough jobsA major part of your job is to help the pa-tient manage his symptoms in a way thatwill best preserve his quality of life. Fol-lowing are some guidelines for symptommanagement.n Monitor headache onset, intensity, andsymptoms. Advise the patient about med-ications, like steroids and analgesics, thatwill relieve the pain.

n Monitor seizure activity and the type ofseizure. Instruct the patient about appro-priate antiseizure medications, includingadministration and adverse effects. Seealso Brainstorm: Managing a seizure.n The patient with a brain tumor may ex-perience significant fatigue from the tumor


Plan BStandard treatment sometimes falls short of improving the 5-year survival rate for certain tumor types. According to the NationalCancer Institute, new biologic therapies being looked at include dendritic cell vaccination, tyrosine kinase receptor inhibitors, far-nesyl transferase inhibitors, viral-based gene therapy, and oncolytic viruses. Patients with tumors that are unresectable or infre-quently curable may benefit from radiosensitizers, hyperthermia, or interstitial brachytherapy used along with external beam radi-ation therapy to boost its benefit. Following are some of the other new treatment options in clinical trials.

Tumor type Standard treatment Treatment options under study

Pilocytic astrocytomas Surgery or surgery and radiation Nitrosourea-based chemotherapy, temozolomide

Anaplastic astrocytomas Surgery plus radiation or surgery plus Hyperfractionated irradiation, accelerated-fractionradiation and chemotherapy irradiation, stereotactic radiosurgery

Glioblastoma Surgery plus radiation or surgery plus Hyperfractionated irradiation, accelerated-fractionradiation and chemotherapy, carmustine irradiation, stereotactic radiosurgery(BCNU)-impregnated polymer (Gliadel wafer), radiation plus temozolomide

Oligodendroglial Surgery or surgery plus radiation Temozolomide, procarbazine-lomustine-vincristine (PCV) therapy

Brainstorm: Managing a seizure

• Have the patient lie down immediately if hefeels a seizure coming on. Some patients willhave a warning symptom, like a visual aura or asmell.

• Loosen clothing around the neck andabdomen.

• Clear away all objects close to the patientthat could injure him.

• Stay with the patient to provide support andto document manifestations of the seizure.

• Ensure airway patency. Never force apatient’s mouth open during a seizure.

• Once the seizure has subsided, providereassurance and reorient the patient to time,place, and person.

itself or from the treatments.Advise him to get adequate

rest, food, and fluids. Con-sult with a dietitian if nausea

and vomiting or anorexia make it im-possible for the patient to take inproper nutrition. n The patient may experience cogni-

tive impairment and/or memory loss. Theprovider may order medications that willreduce these distressing symptoms.

Providing plenty of good, understand-able information so that the patient canmake informed decisions about his care isone of the best ways you can help him copewith a diagnosis of brain tumor and itsaftermath. “Is there anything you’d like toask?” can be a key phrase in avoiding mis-apprehension. Often, you can use his ques-tions as an indication of how much infor-mation he really wants to know.

Be on the lookout for symptoms of majordepression in a long-term patient. Once the

hectic back-and-forth of tests and chemotreatments disappears, the full implicationsof having a brain tumor may finally sink inand be overwhelming. Refer the patientand his family to hospital social services,which will provide them with informationon the many brain tumor support groupsavailable. n

Learn more about itArmstrong TS, et al. Seize the moment to learn aboutepilepsy in people with cancer. Clinical Journal of OncologyNursing. 7(2):163-169, March-April 2003.

National Cancer Institute. Adult brain tumors (PDQ):Treatment: Health Professional Version. http://www.nci.nih.gov/cancertopics/pdq/treatment/adultbrain/healthprofessional/allpages. Accessed February 17, 2006.

Ree A, et al. Direct infiltration of brainstem glioma alongthe cranial nerves. Journal of Neuroimaging. 15(2):197-199,April 2005.

Smeltzer SC, Bare BG. Brunner & Suddarth’s Textbook ofMedical-Surgical Nursing, 10th edition. Philadelphia, Pa.,Lippincott Williams & Wilkins, 2004.

Whittle IR, et al. Gliadel therapy given for first resectionof malignant glioma: A single centre study of the poten-tial use of Gliadel. British Journal of Neurosurgery.17(4):352-354, August 2003.


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Brain tumors - Lippincott Williams & Wilkins...United States, brain tumors are the deadliest of childhood cancers. And just because a brain tumor is benign doesn’t mean it’s harmless;