$Unique_ID{BRK03538} $Pretitle{} $Title{Brain Tumors, General} $Subject{Brain Tumors General Intracranial Tumors Primary Tumors of Central Nervous System Benign and Malignant Tumors of CNS Benign Brain Tumors Malignant Brain Tumors Cluster Headache Tolosa-Hunt Syndrome Hydrocephalus Pseudotumor Cerebri Meningitis} $Volume{} $Log{} Copyright (C) 1991, 1992, 1993 National Organization for Rare Disorders, Inc. 813: Brain Tumors, General ** IMPORTANT ** It is possible that the main title of the article (Brain Tumors, General) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Intracranial Tumors Primary Tumors of Central Nervous System Benign and Malignant Tumors of CNS Disorder Subdivisions: Benign Brain Tumors Malignant Brain Tumors Information on the following diseases can be found in the Related Disorders section of this report: Cluster Headache Tolosa-Hunt Syndrome Hydrocephalus Pseudotumor Cerebri Meningitis 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. Brain Tumors are abnormal growths in the brain that can be either cancerous (malignant) of noncancerous (benign). The effects on the brain of malignant and benign brain tumors are very similar and can cause the same types of problems depending upon the type of tumor and where it is located in the brain. Symptoms Brain Tumors are characterized by symptoms that can often be confused with other disorders. Chronic headaches, behavior and thought changes, convulsions, vomiting and swelling of the eyes (papilledema) are a few of the common symptoms. Other neurological signs that may indicate the existence of a tumor include difficulties or changes in language, ataxia (unsteady walking), sensory impairment and weakness. Brain tumors produce generalized symptoms because they cause swelling and compression in specific areas of the brain. Diagnosis is usually made by a neurologist using specific diagnostic imaging tests such as CT (Computed Tomographic scans) or MRI (Magnetic Resonance Imaging scans) of the patient's brain. Benign Brain Tumors are usually slow growing and do not cause the death of brain cells and vascular spreading that malignant brain tumors do. There are many different types of benign tumors such as astrocytomas, mesodermal meningioma, neuroectodermal oligodendrogliomas and some forms of pineal tumors, etc. However, even though these types of tumors are most often benign they can cause very serious complications if they are not completely removed. Malignant Brain Tumors consist of medulloblastomas, anaplastic astrocytomas, Glioblastoma multiforme, vascular tumors, some pituitary tumors, etc. Adenomas, metastatic tumors and primary lymphomas are usually highly malignant. They may originate in the brain or spread to the brain from other cancers elsewhere in the body. Causes The exact causes of brain tumors are unknown. Some are thought to be genetically transmitted, others are congenital (present at birth), and still others develop later as a result of cancer in other parts of the body that spreads to the brain and central nervous system. Others occur for no apparent cause. Affected Population Brain Tumors are not rare, although each subtype of tumor can be relatively rare. They affect males and females in equal numbers. Brain tumors can occur in children as well as adults. Malignant brain tumors are the second most common cancer in children, and in adults they are more common than Hodgkin's disease. Related Disorders Symptoms of the following disorders can be similar to those of Brain Tumors. Comparisons may be useful for a differential diagnosis: Cluster Headaches are a rare form of severely disabling headache. The headache is a deep, non-throbbing, extremely painful one which tends to recur in the same area of the head or face with each occurrence. They usually come on during sleep and awaken the patient. They are typically associated with watering of the eyes and nose. (For more information on this disorder, choose "Cluster Headache" as your search term in the Rare Disease Database). Tolosa-Hunt Syndrome is a rare disorder. It includes chronic headaches, mild fever and vision impairment followed by painful eye muscle paralysis. Swelling, protrusion of the eye, drooping eyelid, diminished vision and abnormal skin sensations around the eye may be associated with the paralysis. These symptoms usually occur only on one side of the head. Additionally, symptoms often associated with migraine headaches such as double vision, nausea, vomiting and a general feeling of discomfort may develop. (For more information on this disorder, choose "Tolosa-Hunt" as your search term in the Rare Disease Database). Hydrocephalus is characterized by swelling of the brain. This is caused by the abnormal dilation of the ventricles (cerebral spaces) in the brain and the obstruction of the spinal fluid passages (ventricular block) of the central nervous system. This accumulation of fluid puts excessive pressure on the tissues of the brain, and causes an enlargement of the head. Hydrocephalus may be a congenital or an acquired disorder, and, if not treated, may cause brain damage. Headache, vomiting, weakness, giddiness, uncoordinated movements, and eventually mental deterioration may occur. Hydrocephalus may occur in conjunction with a brain tumor, or without any tumor. (For more information on this disorder, choose "Hydrocephalus" as your search term in the Rare Disease Database). Meningitis is a rare inflammation of the membranes of the brain and spinal cord. It can be caused by bacteria, viruses, and other infectious organisms. The infection reaches the membranes (Meninges) of the brain through the blood or Lymph system causing intense headache. (For more information on this disorder, choose "Meningitis" as your search term in the Rare Disease Database). Pseudotumor Cerebri is a rare syndrome of increased pressure inside the skull. The symptoms include headache of varying degrees of severity which are unrelieved by medication. A swelling of the optic disc (papilledema) with progressive visual loss occurs in a small percentage of the patients. Occasionally, there is a loss of strength and energy, and memory disturbances that may interfere with daily life. (For more information on this disorder, choose "Pseudotumor Cerebri" as your search term in the Rare Disease Database). Therapies: Standard Treatment of Brain Tumors, whether they are malignant or benign, usually consists of surgery to remove as much of the tumor as is possible without causing damage to the surrounding tissue. Surgery is often followed or accompanied by radiation and/or chemotherapy treatments. Therapies: Investigational There are many experimental treatments for brain tumors which are usually tried when other standard therapies fail. Some primary malignant Brain Tumors are being treated with experimental orphan drugs such as Interferon alfa-2b (recombinant). Serratia marcescens extract (polyribosomes) is another experimental orphan drug being used in the treatment of primary brain malignancies. The drug adenosine is being researched as a treatment for some types of brain tumors as is the polymer implant containing biodegradable carmustine (Biodel) which is placed in the brain for treatment of recurrent malignant glioma. Glioblastoma multiforme is usually treated with photon therapy. An alternative to that therapy is the experimental orphan drug Borolife (sodium monomercaptoundecahydro-closo-dodecaborate). Some types of brain tumors are treated with implanted radioactive therapies. All of these investigational treatments require more research before they can be proven safe and effective. Lynn Hayes has received a New Grant Award from the Office of Orphan Products for her studies of Phase 1/11: IL-2/LAK for Primary, Pediatric Brain Tumors. Dr. Hayes is with New York University Medical Center, New York, NY. To learn more about experimental treatments of brain tumors contact the Brain Tumor Research Association, listed in the resources section of this report. Liposome Encapsulated Recombinant Interleukin-2 is a new orphan product being developed by Oncotherapweutics, Inc., 527 Marquette Ave., 9th Fl., Minneapolis, MN, 55402. It is used to treat brain and CNS tumors. An important breakthrough in the treatment of AVM's and deep-seated brain tumors is the use of the Gamma Knife, a Swedish-made machine. The machine uses gamma rays from 201 cobalt-60 radioactive sources to destroy swollen brain arteries and both malignant and benign brain tumors. This machine, located at the University of Pittsburgh Medical Center, is the first and only one of its type in North America. The success rate for treatment of both AVM's and brain tumors is very impressive. The orphan drug Interferon Beta, Recombinant Human, is being tested as a treatment for brain tumors. The drug is sponsored by: Biogen, Inc., 14 Cambridge Center, Cambridge, MA, 02142. This disease entry is based upon medical information available through February 1993. 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 General Brain Tumors, please contact: National Organization for Rare Disorders P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 The American Brain Tumor Association 3725 North Talman Ave. Chicago, IL 60618 (800) 886-2282 American Cancer Society 1599 Clifton Rd., NE Atlanta, GA 30329 (404) 320-3333 NIH/National Cancer Institute (NCI) 9000 Rockville Pike, Bldg. 31, Rm. 1A2A Bethesda, MD 20892 1-800-4-CANCER The National Cancer Institute has developed PDQ (Physicians 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 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 CECIL TEXTBOOK OF MEDICINE, 18th Ed.: James B. Wyngaarden, and Lloyd H. Smith, Jr., Editors; W.B. Saunders Co., 1988. Pp. 2229-2238. STEREOTAXIC IRRADIATION OF BRAIN TUMORS., D.A. Larson, et al,; Cancer, (February 1, 1990, issue 65 (3 suppl)). Pp. 792-799. INTRACRANIAL EPENDYMOMA AND SUBEPENDYMOMA: MR MANIFESTATIONS. G.P. Spoto, et al.; AJR Am J Roentgenol, (April, 1990, issue 154 (4)). Pp. 837- 845. CT AND MR FINDINGS IN PATIENTS WITH CHORDOMAS OF THE PETROUS APEX. R.V. Brown, et al,; AJNR (January-February, 1990, issue 11 (1)). Pp. 121-124.