Ependymoma

Definition

A brain tumor is an abnormal growth of tissue in the brain. Unlike other tumors, brain tumors spread by local extension and rarely metastasize (spread) outside the brain. A benign brain tumor is composed of non-cancerous cells and does not metastasize beyond the part of the brain where it originates. A brain tumor is considered malignant if it contains cancer cells, or if it is composed of harmless cells located in an area where it suppresses one or more vital functions.

Description

Each year, more than 17,000 brain tumors are diagnosed in the United States. About half of all brain tumors are benign, but in life-threatening locations. The rest are malignant and invasive.

Benign brain tumors

Benign brain tumors, composed of harmless cells, have clearly defined borders, can usually be completely removed, and are unlikely to recur. Benign brain tumors do not infiltrate nearby tissues but can cause severe pain, permanent brain damage, and death. Benign brain tumors sometimes become malignant.

Malignant brain tumors

Malignant brain tumors do not have distinct borders. They tend to grow rapidly, increasing pressure within the brain (IICP) and can spread in the brain or spinal cord beyond the point where they originate. It is highly unusual for malignant brain tumors to spread beyond the central nervous system (CNS).

Primary brain tumors

Primary brain tumors originate in the brain. They represent about 1% of all cancers and 2.5% of all cancer deaths.

Metastatic or secondary brain tumors

Approximately 25% of all cancer patients develop secondary or metastatic brain tumors when cancer cells spread from another part of the body to the brain. Secondary brain tumors are most apt to occur in patients who have:

• Breast cancer
• Colon cancer
• Kidney cancer
• Lung cancer
• Melanoma (cancer) of the skin. These metastatic brain tumors can develop on any part of the brain or spinal cord.

Who gets brain tumors

Brain tumors can develop at any age, but are most common in children between the ages of 3-12, and in adults aged 40-70. Primary brain cancer is the second most common cause of cancer death between birth and the age of 34, and the third most common cause of cancer death in men aged 35-54. Primary tumors of the brain and central nervous system are often associated with HIV infection.

Naming and grading brain tumors

The name of a brain tumor describes where it originates, how it grows, and what kind of cells it contains. A tumor in an adult is also graded or staged according to:

• How malignant it is
• How rapidly it is growing and how likely it is to invade other tissues
• How closely its cells resemble normal cells. (The more abnormal a tumor cell looks, the faster it is likely to grow).

Low-grade brain tumors usually have well-defined borders. Some low-grade brain tumors form or are enclosed (encapsulated) in cysts. Low-grade brain tumors grow slowly, if at all. They may spread throughout the brain, but rarely metastasize to other parts of the body.

Mid-grade and high-grade tumors grow more rapidly than low-grade tumors. Described as "truly malignant," these tumors usually infiltrate healthy tissue. The growth pattern makes it difficult to remove the entire tumor, and these tumors recur more often than low-grade tumors.

A single brain tumor can contain several different types of cells. The tumor's grade is determined by the highest-grade (most malignant) cell detected under a microscope, even if most of the cells in the tumor are less malignant. An infiltrating tumor is a tumor of any grade that grows into surrounding tissue.

Types of brain tumors

Glioma is the term used to refer to the most prevalent primary brain tumors. Gliomas arise from glial tissue, which supports and nourishes cells that send messages from the brain to other parts of the body. These tumors may be either malignant or benign. Astrocytomas, ependymomas, and mixed gliomas are three of the most common gliomas.

Named for the star-like shape of their cells, astrocytomas can develop on any part of the brain or spinal cord. Non-infiltrating astrocytomas grow slowly, and rarely spread to nearby tissue. Mild-to-moderately anaplastic astrocytomas with well-differentiated borders do not grow as slowly as non-infiltrating astrocytomas, and they do spread to surrounding tissues.

Anaplastic astrocytomas, which are also called Grade III astrocytomas, look more abnormal and grow more rapidly than non-infiltrating or mild-to-moderately anaplastic tumors.

Grade IV astrocytomas are also called glioblastoma multiforme (GBM) tumors. Accounting for 30% of all primary brain tumors, GBMs are the most common brain tumors in middle-aged adults. GBMs are the most malignant of all brain tumors. Because they contain a greater mixture of cells than any other brain tumor, they are the most difficult to treat.

Also called ependymal tumors, ependymomas account for 9% of all gliomas, and 5% of all intracranial tumors. These tumors, which are most common in children and adolescents, begin in the very thin membranes that help form cerebrospinal fluid (CSF) and line the brain cavities (ventricles) that contain it.

Ependymomas are usually benign, have well-differentiated borders, resemble normal cells, and grow very slowly. The cells of anaplastic (malignant) ependymomas look abnormal and grow more rapidly than the cells of benign tumors.

These heterogeneous tumors contain elements of astrocytomas and ependymomas and/or oligodendrogliomas. These are rare tumors that usually occur in middle-aged adults, grow slowly, and do not usually spread beyond the part of the brain where they originate. Mixed gliomas behave like tumors composed of the highest-grade cells they contain.

Non-glial brain tumors

The most common brain tumors that do not develop from glial cells are medulloblastomas, meningiomas, and Schwannomas.

Scientists once thought medulloblastomas (MDLs) developed from glial cells. These fast-growing, malignant tumors are now believed to originate in developing cells not normally present in the body after birth. They are sometimes called primitive neurodectal tumors (PNET).

MDL tumors are most common in children and are more common in boys than in girls. Only 30% of MDL tumors occur in adults. MDL tumors usually originate in the cerebellum (the part of the brain that controls coordination and some muscle activity), and are often carried to other parts of the brain by cerebrospinal fluid. MDL tumors rarely metastasize beyond the brain and spinal cord.

Meningiomas, which represent more than 20% of all primary brain tumors, originate in the membranes that enclose the brain and spinal cord (meninges). These tumors are usually benign and most often occur in women aged 30-50 years old. Meningiomas grow so slowly that the brain can sometimes become accustomed to their presence. Meningiomas compress, rather than invade, brain tissue and may grow to be quite large before any symptoms appear.

Schwannomas originate in the Schwann cells. These cells produce myelin, material that protects the acoustic nerve, which controls hearing. These benign tumors are twice as common in women as in men, and are most often diagnosed in patients between the ages 30-60.

Schwannomas grow very slowly, and many people adapt to the slight hearing loss and balance problems that are the tumors' earliest symptoms. A pear-shaped Schwannoma can cause sudden or gradual loss of hearing in an ear. As the tumor progresses, it can press on the nerves that control movement and feeling in the face, and cause headaches and facial numbness or tingling. The patient may have trouble walking, swallowing, or controlling eye movements, and the sense of taste can be affected. A Schwannoma that grows large enough to press on the brainstem can be deadly.

Brain tumors that occur in children are described as supratentorial (in the upper part of the brain) or infratentorial (in the lowest part of the brain). Astrocytomas and ependymomas are common supratentorial tumors. Infratentorial tumors include medulloblastomas, astrocytomas, and ependymomas.

Causes & symptoms

The cause of primary brain tumors is unknown, but people who work with rubber and certain chemicals have a greater-than-average risk of developing them. There is no evidence that head injury causes brain tumors, but researchers are trying to determine the relationship, if any, between brain tumors and viruses, family history, and long-term exposure to electromagnetic fields.

Symptoms do not usually appear until the tumor grows large enough to displace, damage, or destroy delicate brain tissue. When that happens, the patient may experience:

• Headaches that become increasingly painful and are most painful when lying down
• Nausea and vomiting or sudden attacks of vomiting not accompanied by nausea
• Seizures
• Dizziness, loss of coordination or balance
• Personality changes
• Sudden loss of vision
• Memory loss
• Speech problems
• Sensory changes
• Mental impairment
• Weakness or paralysis on one side of the body.

A doctor should be notified whenever a patient experiences one or more of the symptoms.

Diagnosis

Although brain tumor symptoms resemble those of many other illnesses, the presence of a brain tumor may be indicated by:

• Persistent headaches with vomiting or convulsions
• Progressive deterioration of sight, speech, hearing, touch; or deterioration in the ability to use an arm, hand, foot, or leg.

When a patient experiences one or more of the above symptoms, a primary care physician will perform a complete physical examination, take a detailed medical history, and conduct a basic neurologic examination to evaluate:

• Balance and coordination
• Abstract thinking and memory
• Eye movements
• Hearing, touch, and sense of smell
• Reflexes
• Control of facial muscles and movements of the head and tongue
• Awareness.

If the results of these examinations suggest a patient may have a brain tumor, a neurologist recommends some or all of these additional diagnostic tests:

• Computed tomography scan (CT scan) to reveal brain abnormalities
• Magnetic resonance imaging (MRI) to detect tumors beneath the bones of the skull
• Electroencephalography (EEG) to measure electrical activity in the brain
• X rays to reveal any distortion in the bones of the skull
• Angiography to outline a tumor and the blood vessels that lead to it
• A brain scan to identify and record the location of abnormal cells in the brain
• Myelography (x ray of the spine) to detect a spinal cord tumor
• A lumbar puncture (spinal tap) to obtain spinal fluid, which may contain tumor cells.

Interpreting these images and results of laboratory analysis allows neurologists to determine whether a tumor is present, but microscopic examination of tumor tissue (biopsy) is the only way to identify the kind of cells it contains.

Treatment

Brain tumors are treated by multidisciplinary teams of highly skilled specialists whose decisions are based on:

• Results of diagnostic tests
• Tumor size, position, and growth pattern
• The patient's health history and current medical status
• The wishes of the patient and his family.

Surgery

Surgery is the treatment of choice for accessible brain tumors, which can be removed without causing serious neurologic damage. The procedure most often performed is a craniotomy, but the goals of any type of brain tumor surgery include:

• Removing as much of the tumor as possible
• Removing tumor tissue for microscopic analysis
• Allowing neurosurgeons to see exactly how the tumor is situated and how it is growing
• Creating an entry channel for chemotherapy drugs and forms of radiation that are implanted in the brain.

Before undergoing brain surgery, patients are often given:

• Steroids to reduce swelling of brain tissue
• Anticonvulsant medications to prevent or control seizures
• Radiation treatments to reduce tumor size.

Patients whose benign brain tumors can be completely removed may not require any additional treatment, but periodic physical and neurologic examinations and CT or MRI scans are sometimes recommended to determine whether the tumor has returned. Because surgeons cannot be sure that every bit of an infiltrating or metastasizing tumor has been removed, radiation and chemotherapy are used to eradicate cells that may have escaped the scalpel.

If a tumor cannot be completely removed, removing a portion of it (debulking) can alleviate the patient's symptoms, enhance the sense of well-being, and increase the effectiveness of other treatments.

Radiation therapy

External radiotherapy, generally delivered on an outpatient basis, directs radiation to the tumor and the area around it. Implant radiation therapy involves placing tiny pieces of radioactive material in the brain. Left in place permanently, or for a short time, these radioactive pellets release measured doses of radiation each day. Patients are usually hospitalized during the several days the pellets are most active.

Stereoactic radiosurgery involves fitting the patient with a frame to stabilize the head, using imaging techniques to determine the exact location of tumor cells, and using a sophisticated instrument called a gamma knife to administer radiation precisely to that point.

Chemotherapy

One or more cancer-killing drugs may be taken by mouth or injected into a blood vessel, muscle, or the cerebrospinal fluid. Chemotherapy may be used with radiation and surgery as part of a patient's initial treatment, or used alone to treat tumors that recur in the same place or in another part of the body.

When a young child has a brain tumor, chemotherapy is often used to eliminate or delay the need for radiation.

Other treatments

If a brain tumor cannot be cured, treatment is designed to make the patient as comfortable as possible and preserve as much of his neurologic functioning as possible. The patient's doctor may prescribe:

• Analgesics to relieve pain
• Anticancer drugs to limit tumor growth
• Anticonvulsants to control seizures
• Steroids to reduce swelling of brain tissue.

Potential therapies

Scientists are studying ways to empower chemotherapy drugs to penetrate the blood-brain barrier (which protects the CNS by separating the brain from blood circulating throughout the body), and attack cancer cells that have infiltrated tissue inside it.

Brain tumor researchers are also investigating:

• Less invasive surgical procedures
• Methods of incorporating chemotherapy drugs into tumor cells to reduce the need for radiation
• Laboratory techniques that enable physicians to select the chemotherapy drugs most likely to kill particular types of tumors
• Gene therapy in which genetically engineered material is transported to tumor cells by viruses that infect tumor cells and convert them to normal cells, stop their growth, or kill them.

Alternative treatment

Alternative treatments have not been shown to cure brain tumors and should never be substituted for conventional therapy. However, complementary therapies (used with, not instead of, standard treatments) can help some patients cope with the stress of their illness and side effects of their treatment.

Biofeedback can teach patients to influence and control heart rate, muscle tension, and other stress-related body functions. Some patients claim that guided imagery (visualization) helps them feel healthier and more in control of their disease.

Massage, meditation, and reflexology help some patients relax; while yoga is said to soothe the body, spirit, and mind. Hydrotherapy uses ice, liquid, and steam to improve circulation and relieve pain. Therapeutic touch practitioners say they can relieve pain and other symptoms by moving their hands in slow, rhythmic motions several inches above the patient's body.

Botanical therapies, homeopathic treatment, traditional Chinese medicine treatments, nutritional focuses on diet and supplements, and detoxification can also be incorporated as complementary therapies.

Prognosis

The patient's prognosis depends on where the tumor is located and what type of cells it contains. A patient whose tumor is discovered early and removed completely may make a full recovery, but the surgery itself can harm or destroy normal brain tissue and cause:

• Problems with thought, speech, and coordination
• Seizures
• Weakness
• Personality changes.

Although these post-operative problems may initially be more severe than the symptoms produced by the tumor, they will probably diminish or disappear in time.

Occupational therapy can teach patients and their families new ways to approach daily tasks. Physical therapy can benefit patients who have difficulty keeping their balance, expressing their thoughts, speaking, or swallowing. Children may need special tutors before and after returning to school. For patients who have incurable brain tumors, hospice care may be available. Hospices provide a supportive environment and help patients manage pain and remain comfortable.

Consequences of radiation therapy

Cells killed by radiation can cluster in the brain, resembling tumors. They can cause headaches, seizures, and memory loss. Children treated with radiation may lose some of their eyesight and develop learning problems. Radiation damage to the pituitary gland can hinder normal growth and development.

Consequences of chemotherapy

Some drugs used to treat brain tumors can cause kidney damage and temporary or permanent tingling in the fingers and ringing in the ears.

Inoperable tumors

Brain tumors that cannot be removed may cause irreversible brain damage and death.

Prevention

The cause of primary brain tumors has not been determined, so there is no known way to prevent them.

The best way to prevent secondary or metastatic brain tumors is to eliminate such risk factors as:

• Poor nutrition and a low-fiber diet; since these contribute to development of intestinal cancers
• Smoking, which causes lung cancer
• Excessive use of alcohol, which is associated with liver cancer
• Excessive exposure to the sun, which can cause melanoma (a deadly form of skin cancer).

Monthly self-examinations of the breasts and testicles can detect breast and testicular cancer at their earliest, most curable stages.

Key Terms

Central nervous system (CNS)

The division of the nervous system that consists of the brain and spinal cord.

Cerebrospinal fluid (CSF)

Clear liquid that fills brain cavities and protects the brain and spinal cord.

Gamma knife

High-dose radiation treatment for intracranial tumors.

Intracranial

Located within or on the surface of the brain.

Further Reading

For Your Information

Books

• The Editors of Time-Life Books, Inc. The Medical Advisor: The Complete Guide to Alternative and Conventional Treatment. Alexandria, VA: Time-Life, Inc., 1996.

  Periodicals

• Moulignier, Antoine, et al., "Cerebral Glial Tumors and Human Immunodeficiency Virus-1 Infection: More Than a Coincidental Association." Cancer 74, no. 2 (July 15, 1994): 686-87.

  Organizations

• American Brain Tumor Association. 2770 River Road, Des Plaines, IL 60018. (847) 827-9918, (800) 886-2289. http:/www.abta.org.
• Brain Tumor Foundation for Children, Inc. 2231 Perimeter Park Drive, Suite 9, Atlanta, GA 30341. (404) 454-5554.
• Brain Tumor Information Services. Box 405, Room J341, University of Chicago Hospitals, 5841 S. Maryland Avenue, Chicago, IL 60637. (312) 684-1400.
• MedHelp International. 6300 N. Wickham, Suite 130, Box 188, Melbourne, FL 32940. (407) 253-9048. http://www.medhlp.netusa.net/
• National Brain Tumor Foundation. 785 Market Street, #1600, San Francisco, CA 94103. http://www.oncolink.penn.edu/psychosocial.

  Other

• Adult Brain Tumor. http://cancernet.nci.nih.gov/clinpdq/pif/Adult_brain_tumor_Patient.html (12 March 1998).
• Brain Tumor. http://www.thriveonline.com/health/Library/illsymp/illness53.html (12 March 1998).
• Brain Tumors on the Rise. http://www.thriveonline.com/health/Library/CAD/abstract23554.html (12 March 1998).
• Brain Tumors: Their Symptoms, Therapy, and Future Cure. http://mcns10.med.nyu.edu/intro/treatment.html (22 March 1998).
• Childhood Brain Tumor. http://cancernet.nci.nih.gov/clinpdq/pif/Childhood_brain_tumor_Patient.html (12 March 1998).
• A Primer on Brain Tumors. http://neurosurgery.mgh.harvard.edu/abta/primer.htm (12 March 1998).
• Question: What is a Gamma Knife? http://oncolink.upenn.edu/specialty/med_phys/gamma.html (23 March 1998).
• What You Need to Know About Brain Tumors. http://rex.nci.nih.gov/WTNK_PUBS/brain/index.htm (21 March 1998).

Gale Encyclopedia of Medicine. Gale Research, 1999.