$Unique_ID{BRK03501} $Pretitle{} $Title{Astrocytoma, Malignant} $Subject{Astrocytoma Malignant Giant Cell Glioblastoma Anaplastic Astrocytoma Spongioblastoma Multiforme Astrocytoma grades 3-4} $Volume{} $Log{} Copyright (C) 1986, 1988, 1989 National Organization for Rare Disorders, Inc. 277: Astrocytoma, Malignant ** IMPORTANT ** It is possible the main title of the article (Malignant Astrocytoma) is not the name you expected. Please check the SYNONYMS listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Giant Cell Glioblastoma Anaplastic Astrocytoma Spongioblastoma Multiforme Astrocytoma, grades 3-4 General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Malignant Astrocytoma is an infiltrating, primary brain tumor, with tentacles that may invade surrounding tissue. This provides a butterfly-like distribution pattern through the white matter of the cerebral hemispheres. The tumor may invade a membrane covering the brain (the dura), or spread via the spinal fluid through the ventricles of the brain. Spread of the tumor (metastasis) outside the brain and spinal cord is rare. Symptoms Malignant Astrocytoma grows by spreading into normal tissue. The first symptoms of this tumor are commonly those of increased cranial pressure. This pressure results from the inability of the bones of the skull to expand to accommodate the growing tumor. A headache which is not localized and is worse in the morning, may be accompanied by vomiting. Nausea is rarely present. Subtle personality changes may precede these symptoms. Malignant Astrocytoma may occur in any area of the cerebral hemispheres, but are most common in the frontal, temporal and parietal lobes, causing symptoms specific to each location. Frontal lobe tumors usually cause intellectual disabilities, such as memory impairment. Patients with this kind of tumor may show little or no emotions (flat personality effect). Symptoms may also include seizures (convulsions) and paralysis (hemiplegia) on the side of the body opposite the location of the tumor. Temporal tumors initially present fewer symptoms, but may include seizures, language interpretation disturbances, and motor disturbances such as lack of coordination of body parts. Parietal tumors are usually characterized by writing disturbances (agraphia), sensory changes such as tingling sensations (paresthesias), spatial disorientation or loss of awareness of the position of parts of the body, and seizures. Causes The cause of Malignant Astrocytoma, like most brain tumors, is unknown. Cases of familial tumors have been reported, but a hereditary mode of transmission has not yet been proven. Occupational chemical factors have been associated with some tumors such as employment in rubber manufacturing industries, vinyl chloride exposure, and farmers exposed to chemical sprays. It has been suggested that children exposed to lead or who have used barbiturates may be at a higher risk to get Malignant Astrocytoma. Recent research suggests that astrocytoma may be caused by a rare virus, but more studies are needed to prove or disprove this theory. Affected Population Malignant Astrocytoma occurs most often in people between the ages of 48 and 60 years, although it can also affect pre-teenage children. It affects twice as many males as females, and whites more frequently than nonwhites. Males with type A blood appear to be at a higher risk. Sixty-five to seventy percent of patients with Astrocytoma survive 1 year, and forty percent 2 years. Many survive longer. Related Disorders Glioblastoma Multiforme is similar to Malignant Astrocytoma, but it is an even more malignant tumor. (For more information on this disorder, choose "glioblastoma multiforme" as your search term in the Rare Disease database.) Therapies: Standard Treatment for this disorder may include surgery, radiation, or chemotherapy. SURGERY--The treatment of choice for accessible Malignant Astrocytoma tumors is surgery. Accessible tumors are those which can be operated on without causing unacceptably severe damage to of the brain. If the tumor is not accessible, a biopsy and steroid medications to control swelling may be recommended instead of surgical removal. The biopsy results may indicate a tumor that is amenable to other treatment methods. A subtotal decompressive resection, or partial removal of tumor tissue, may be performed to decrease symptoms and also improve the chances for other therapies to be effective. In situations where the tumor has extensively invaded the brain, either of these therapies is used primarily for relief of symptoms. If aggressive surgery or total resection is undertaken, the surgeon will attempt to remove all identifiable tumor, often using an operating microscope to better see tumor margins. In some cases, a laser and/or ultrasonic aspirator is used as well. Laser microsurgery has the advantage of being able to remove, by vaporization, some tissue beyond the tumor's border with the hope of removing microscopic tumor infiltrates with a minimal amount of damage to normal tissue. Astrocytomas frequently cannot be totally removed because the spreading tumor, too small for the surgeon to see, is usually present in the surrounding area. Aggressive resection reduces the number of tumor cells to a level where radiation therapy and chemotherapy can be more effective. If the tumor recurs, a second or even third operation may be performed. RADIATION--External radiation is usually recommended following surgery. It begins almost immediately, both to the tumor and to the entire brain. Whole brain irradiation is administered because the Astrocytoma tends to infiltrate widely. After radiation has reduced the number of tumor cells, chemotherapy is administered in an attempt to destroy any cells that remain. Chemotherapy may also be given during the course of radiation treatment. CHEMOTHERAPY--The drugs used in chemotherapy are cytotoxins, or cell poisons, which are capable of destroying cells. Cytotoxins are not completely tumor-cell specific, so they may also cause damage to normal tissue. The type of chemotherapeutic drug selected is determined by a neurooncologist who examines the grade of tumor, previous treatment and current health status of the individual with Malignant Astrocytoma. A neuro- oncologist is a physician who has been specially trained and has experience in treating brain tumors. The most commonly used drugs are BCNU and CCNU. Other drugs may be prescribed on an experimental basis. Therapies: Investigational One of the new experimental techniques for treatment of Malignant Astrocytoma is brachytherapy, also called "interstitial radiation" or "seeding". Via a surgical procedure, radioactive pellets of as Iodine, Iridium or Gold isotopes are implanted directly into the tumor. Brachytherapy is used primarily in recurrences when the tumor is confined to one side of the brain and measures less than 2 1/2 inches (about the size of an egg). Other investigational therapies include use of 1) cell radiosensitizers to increase the effectiveness of radiation; 2) different types of radiation such as neutrons, heat (hyperthermia) and light (photoradiation); 3) intraoperative radiation; and 4) hyperfractionation. These treatments are ongoing research projects which are being clinically tested against Malignant Astrocytoma cells. Immunotherapy aims to stimulate the body's defenses against the tumor. Using drugs such as interferon, levamisole, interleukin-2 and thymosine, and BCG, it is hoped that the body's own immune system can be stimulated to fight the tumor. A multitude of new drugs and drug combinations are being tested for effectiveness against Astrocytoma. Other research seeks to develop better methods of drug delivery, such as direct intra-arterial administration and blood brain barrier disruption to increase the amount of anticancer drug reaching brain tissue. A new orphan drug and delivery system is being tested for the treatment of Astrocytoma and Glioblastoma. During surgery to remove the brain tumor, a biodegradable wafer containing a cancer fighting drug (BCNU) is implanted at the sight of the tumor. At least 220 patients are needed for a clinical trial of this drug. Those interested should have their physician contact Dr. James Kenealy, Nova Pharmaceutical Corp., 6200 Freeport Centre, Baltimore, MD 21224 or phone 301-522-7000. This disease entry is based upon medical information available through June 1989. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Malignant Astrocytoma, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 Association for Brain Tumor Research 2910 West Montrose Ave. Chicago, IL 60618 (312) 286-5571 American Cancer Society 1599 Clifton Rd., NE Atlanta, GA 30329 (404) 320-3333 NIH/National Institute of Neurological Disorders & Stroke (NINDS) 9000 Rockville Pike Bethesda, MD 20892 (301) 496-5751 (800) 352-9424 NIH/National Cancer Institute (NCI) 9000 Rockville Pike, Bldg. 31, Rm. 1A2A Bethesda, MD 20892 (800) 4-CANCER The National Cancer Institute has developed PDQ (Physician Data Query), a computerized database designed to give the public, cancer patients and families, and health professionals quick and easy access to many types of information vital to patients with this and many other types of cancer. To gain access to this service, call: Cancer Information Service (CIS) 1-800-4-CANCER In Washington, DC and suburbs in Maryland and Virginia, 636-5700 In Alaska, 1-800-638-6070 In Oahu, Hawaii, (808) 524-1234 (Neighbor islands call collect) References ABOUT GLIOBLASTOMA MULTIFORME AND MALIGNANT ASTROCYTOMA; D. P. Hesser, et. al., eds.; Association for Brain Tumor Research, 1985.