oli god end ro glioma

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Sharing knowledge. Sharing hope.

F O C U S I N G O N T U M O R S

Oligodendrogliomaand Oligoastrocytoma

American Brain Tumor Association

River Road, Suite Des Plaines, Illinois

.. TEL [email protected] EMAIL

.. FAX www.abta.org WEB American Brain Tumor Association



IntroductionThis publication is about oligodendrogliomaand oligoastrocytoma (tumors which are a mix

of oligodendroglioma and astrocytoma). If yourquestions are about another type of tumor, pleasecall us for a copy of A Primer of Brain Tumors.

“Glial” tissue is the supportive, or nourishing,tissue of the brain. Any tumor of this tissue iscalled a glioma. There are several types of gliomas.

Oligodendrogliomas are gliomas that arise from

oligodendrocytes — fried egg shaped cellswithin the brain. These cells normally formthe covering layer of nerve fibers in the brain.

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A Word About ABTAFounded in 1973, the not-for-profit American

Brain Tumor Association has a proud history

of funding research, providing patient services,and educating people about brain tumors. Ourmission is to eliminate brain tumors throughresearch and meet the needs of brain tumorpatients and their families.

We gratefully acknowledge Deneen PotterHesser RN, BS, OCN for the writing of this

publication. We also appreciate the volunteerefforts of Nina A. Paleologos, MD, AssistantProfessor of Neurology, NorthwesternUniversity Medical School and Co-Director,Neuro-Oncology Program, Evanston Hospital,Evanston, Illinois for technical review. Wealso express our thanks to M. Anne Edwardsfor the original edition of this publication.

This publication was made possiblethrough the generosity of: BSI Ltd. inhonor of Mark N. Nagan; the Doran Family;Robert Richards; Mr. & Mrs. Jay Schroeder;Chelle S. Lancaster’s family honoring herremarkable spirit and positive attitudeduring treatment for her oligodendroglioma;

Stephen D. West; and donations inmemory of Heidi Schechter Moldo andRobert Jon Riechers, MD.

ISBN 0-944093-60-4

COPYRIGHT © 1988. 1989, 1992, 1996, 2001 ABTA

REPRODUCTION WITHOUT PRIOR WRITTEN PERMISSION IS PROHIBITED.

A S T R O C Y T E

Astrocytomas are gliomas that arise fromastrocytes — star-shaped cells within thebrain. Astrocytes store information andnutrients for nerve cells in the brain.

Oligoastrocytomas are “mixed gliomas”containing both oligodendroglioma andastrocytoma cells.

Oligodendrogliomas are generally soft,

greyish-pink tumors. They often containmineral deposits (called calcifications), areasof hemorrhage, and/or cysts. These tumors may be “graded” to describe the appearance of theircells when viewed under a microscope. Usingthe World Health Organization classificationsystem, oligodendrogliomas and oligoastrocytomascan be either grade II or grade III tumors.

O L I G O D E N D R O C Y T E



The cells of a grade II tumor are referred to as

“well-differentiated” — they appear slightly abnormal when compared to normal cells.These tumor cells reproduce at a slow rate.Grade III tumors are “anaplastic” tumors. Thecells of these tumors are definitely abnormal inappearance. Anaplastic tumors tend to containmany blood vessels and cells capable of quickly reproducing. Some anaplastic oligoastrocytomas

contain glioblastoma cells which are grade IV,aggressive cells.

IncidenceAbout 4% of primary brain tumors areoligodendrogliomas, representing about

10-15% of the gliomas. Only 6% of thesetumors are found in infants and children.However, these tumors may be more commonthan generally thought since newer biologicmarkers now help pathologists separateoligodendrogliomas from other types of tumors. Most oligodendrogliomas occur inadults ages 50-60, and are found in men more

often than than women.

CauseThe cause of these tumors, as well as othertypes of brain tumors, is unknown. Scientistshave identified abnormalities on chromosomes 1p

and 19q which may play a role in the developmentof oligodendroglioma and oligoastrocytoma. Inaddition, anaplastic tumors appear to haveabnormalities on chromosome 9 or 10, alongwith unusual amounts of growth factors and geneproteins. Those substances are thought to controlthe growth of blood vessels around a tumor.

Researchers believe that both oligodendrogliomasand astrocytomas may originate from one mothercell whose “offspring” follow two differentdevelopmental pathways. This research may help explain the biologic relationship betweenthe two tumor types. However, the initialsteps which change these cells from normalto abnormal still are uncertain.

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C T S C A N O F A O L I G O D E N D R O G L I O M A

CT scan showing an extensively calcified oligodendrogliomalocated in the right temporo-occipital area. The small light areas in the front of both anterior horns are also tumor.

Reprinted from Pathology and Genetics: Tumours of the Nervous System,

IARC Press, Lyon France with permission of Drs. Manfred Westphal,

Paul Kleihues, and Webster Cavenee.

T U M O R



SymptomsBecause of their generally slow growth,oligodendrogliomas are often present for yearsbefore diagnosis. The most common symptomsare seizures, headaches, and personality changes.Other symptoms vary by location and size of the tumor.

These tumors can be found anywhere withinthe cerebral hemispheres of the brain, althoughthe frontal and temporal lobes are the most

common locations. Tumors of the frontal lobemay cause weakness on one side of the body,personality or behavior changes, and difficulty with short term memory. Temporal lobe tumorsare usually “silent,” causing few symptoms otherthan perhaps seizures or language problems.

If you would like to learn more about the

symptoms of brain tumors, please see ourbook, A Primer of Brain Tumors.

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DiagnosisAfter a neurological examination, MRI and/orCT scans are ordered. The calcification some-times present in an oligodendroglioma may beseen on a CT scan and suggests the diagnosis.However, only examination of a sample of tumor tissue by a pathologist confirms the exactdiagnosis and leads to appropriate treatment.


F U N C T I O N S O F T H E L O B E S O F T H E B R A I N

F R O N T A L L O B EP A R I E TA L L O B E

O C C I P I T A L

L O B E

T E M P O R A L L O B E

ThoughtReasoningBehaviorMemory

Sensory PerceptionSpatial Relations

Left: Speech, Motion, SensationRight: Abstract Concepts(For Right-handed Individuals)

Vision

H e a r i n g

C E R E B E L L U M

BalanceCoordination

M E D U L L A

P O N S

M o v e m

e n t

S e n s a t i o

n

BehaviorMemoryHearing & Vision PathwaysEmotion

M R I O F A O L I G O D E N D R O G L I O M A

MRI courtesy of Dr. Paleologos

T U M O R A R E A

Treatment

S U R G E R Y

Surgery is the primary treatment for anoligodendroglioma or oligoastrocytoma if itis located in an accessible area of the brain. An“accessible” tumor is one that can be removedwithout causing severe neurological damage.Numerous tools are available to assist theneurosurgeon in tumor removal. Computer-guided stereotactic navigational systems can



help define the exact tumor location, and brainmapping techniques may help outline vitalparts of the brain to be avoided during surgery.Even with the use of these tools however, sometumors can be only partially removed because of their location. If the tumor is inoperable, a biopsy will be done to confirm the exact diagnosis.

R A D I A T I O N

For an adult with an anaplastic tumor or anoligoastrocytoma, radiation therapy may follow

surgery. If your tumor is an oligodendroglioma,your doctor will determine if radiation therapy is recommended at this time.

There are different types of radiation whichuse various doses and schedules. Most forms of radiation, however, are aimed at the tumor anda small area around the tumor. Conventionalexternal beam radiation is “standard” radiationgiven 5 days a week for 5 or 6 weeks. A formof “local radiation” may be used to boostconventional radiation. Stereotactic radiosurgery aims converged beams of radiation at thetumor. Conformal photon radiation (intensity-modulated radiation therapy) shapes radiationbeams to the shape of the tumor. Interstitial

radiation, also called brachytherapy, may beimplanted into the tumor during surgery.Monoclonal antibodies may be capable ofcarrying radiation or drugs to the tumor site.

C H E M O T H E R A P Y

PCV — the combination of the drugs procarbazine,lomustine (CCNU) and vincristine — has beenfound to be an effective treatment for both

anaplastic oligodendrogliomas and anaplasticoligoastrocytomas. Some physicians also usePCV for lower-grade oligodendrogliomas.About 60-65% of oligodendrogliomas respondto chemotherapy, but scientists are unclear asto which tumors will respond and which willnot. New tests being developed may help betterpredict the effectiveness of chemotherapy in this

group of tumors. In anaplastic oligodendrogliomas,loss of the chromosomes 1p and 19q is strongly associated with chemosensitivity. While mostexperience is with PCV, a newer drug,temozolomide, is being increasingly used inpeople with oligodendroglial tumors.

Chemotherapy may be used in infants and

very young children to delay radiation therapy until the age of three or four. Clinical trials areunderway to evaluate the most effective waysof treating these tumors in infants and children.

There are several drugs used to relieve thesymptoms of a brain tumor. Steroids are drugsused to decrease swelling (edema) around the

tumor. Anti-epilepsy drugs control seizures.Anti-emetic drugs prevent vomiting and helpcontrol nausea. Additional suggestions formanaging side-effects are in our publication, A Primer of Brain Tumors.

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Just as in treating any disease, treatment for a braintumor may have side effects. Ask your doctor to talk with you and your family about these potential effects.He or she can also help you balance the risks oftreatment against the potential benefits.

Some treatments are investigationaland are offered in organized testing

plans called “clinical trials.” Your doctor can tell you if the treatment you areconsidering is a standard treatmentor an investigational treatment.



RecurrenceTumors recur when all the tumor cells cannotbe removed by surgery or killed by other

treatments. Over time, those cells multiply andresult in tumor regrowth. A tumor may recuras a higher grade tumor. It may contain agreater percentage of anaplastic cells, moreastrocytoma cells, or the tumor may spreadwithin the brain or into the spinal canal.However, because many oligodendrogliomasare slow growing tumors, it may be years before

regrowth occurs.

Treatment for a recurrent tumor may beadditional surgery, radiation therapy if thetumor was not previously radiated, or a formof local radiation if the tumor was previously radiated. There are also many clinical trials opento those with a recurrent tumor. Chemotherapy

using drugs such as temozolomide or CPT11;new combinations of drugs; high-dose drugsused with a bone marrow transplantation; orbiodegradable wafers soaked with chemotherapy drugs may all be considered. Anti-angiogenesisdrugs are thought to interfere with the growthof new blood vessels which feed a tumor.Monoclonal antibodies (MOAB) are immune

substances which may be capable of killingcells themselves, or they may serve as a deliveryvehicle for drugs or radioactive products.

Finding Clinical TrialsMany investigational treatments — called clinical

trials — are available to patients with anoligodendroglioma or an oligoastrocytoma. Tofind out more about these studies contact theNational Cancer Institute’s Cancer InformationService at 800-422-6237. They can provideyou with detailed information about brain tumorsas well as a listing of clinical trials specific to your

type of tumor. We maintain a resource listing of physicians participating in clinical trials forbrain tumors, including oligodendrogliomas.Our office can be reached at 800-886-2282.

PrognosisHow well a patient responds to treatment isaffected by their age, location of the tumor,grade of the tumor cells, and the amount of tumor that was able to be removed duringsurgery. Well-differentiated oligodendrogliomas

tend to be lower grade, slow growing tumors.Anaplastic oligodendrogliomas are moreaggressive, faster growing tumors. The outcomefor those with an oligoastrocytoma dependson the percent of astrocytoma versusoligodendroglioma in the tumor, and the mostaggressive type of cell found in the tumor.

Published survival rates for these tumors may not yet reflect the recent positive impact of chemotherapy. If you have an oligodendroglioma,your doctor is the best person to answer questionsabout the expected outcome. He/she can provideyou with information specific to your tumor.When considering a therapy, ask your doctorhow the recommended treatment will affectyour prognosis. What are the expected benefitsof this treatment? What are the risks? Whatquality of life can you expect during and afterthe treatment? If this is an investigationaltreatment, how many patients with your tumortype have received this treatment, and whatwere their results?

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Questions I Want to Ask ResourcesSupport groups and pen-pal programs allow you to share experiences with others in the

same situation. Social workers can help youfind these support networks, as well as sourcesof financial assistance, transportation help,and/or rehabilitation programs. Nurses canprovide you with information about how tocare for yourself or your loved one. Dieticianscan design a healthy eating plan to ensure yourbody receives the nutrients it needs during and

after treatment. We can help you locate theseresources in your area.

A Next Step“Becoming Well Again Through…” is an ABTAquality of life series exploring rehabilitative

medicine, memory retraining, managing fatigue,caregiver stress, and financial aid resources.Please call us at 800-886-2282 for a copy of theseries. Our web site — www.abta.org — offersextensive brain tumor information, treatmentand research updates, and patient/family stories.The thread that runs through each of our servicesand programs is hope. Become involved — join

us in some way, to make sure there is a cure,and ultimately, a way to prevent brain tumors.

We hope that the information in this pamphlethelps you communicate better with the peoplewho are caring for you. Our purpose is not toprovide answers; rather, we encourage you toask questions.




Questions I Want to Ask


Publications & Services

B U I L D I N G K N O W L E D G E

A Brain Tumor — Sharing Hope

Tumor del Cerebro — Compartiendo la EsperanzaDictionary for Brain Tumor PatientsLiving with a Brain TumorA Primer of Brain Tumors

F O C U S I N G O N T U M O R S

EpendymomaGlioblastoma Multiforme and Anaplastic AstrocytomaMedulloblastoma

MeningiomaMetastatic Brain TumorsOligodendroglioma and OligoastrocytomaPituitary Tumors

F O C U S I N G O N T R E A T M E N T

Gene TherapyRadiation Therapy of Brain Tumors: A Basic GuideStereotactic Radiosurgery

F O R & A B O U T C H I L D R E N

Alex’s Journey: The Story of a Child with a Brain Tumor(for ages 9-13, video and booklet formats)

When Your Child Returns to School

S U P P O R T R E S O U R C E S

A Bibliography of Books & ResourcesBrain Tumor Survivor’s Guide to the Internet

Care OptionsEmergency Alert Wallet CardsFinancial Aid ResourcesHousing During Treatment ResourcesOrganizing a Support GroupScholarship & Educational Financial ResourcesSupport Group ListingsTransportation ResourcesWig and Head Covering Resources

Wish Granting Resources

American Brain Tumor Association

River Road, Suite Des Plaines, Illinois

.. TEL [email protected] EMAIL

.. FAX www.abta.org WEB

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