$Unique_ID{BRK03500} $Pretitle{} $Title{Astrocytoma, Benign} $Subject{Astrocytoma Benign Astrocytoma Grade I Astrocytoma Grade II Brain Tumor Intracranial Tumor Intracranial Neoplasm Astrocytoma Malignant Motor Neuron Disease Multiple Sclerosis Brain Tumors} $Volume{} $Log{} Copyright (C) 1990 National Organization for Rare Disorders, Inc. 775: Astrocytoma, Benign ** IMPORTANT ** It is possible that the main title of the article (Benign Astrocytoma) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Astrocytoma Grade I Astrocytoma Grade II Brain Tumor Intracranial Tumor Intracranial Neoplasm Information on the following diseases can be found in the Related Disorders Section of this report: Astrocytoma, Malignant Motor Neuron Disease Multiple Sclerosis Brain Tumors 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. Benign astrocytomas are abnormal growths or tumors which occur in the brain. They are composed of star-shaped neurological cells called astrocytes. Astrocytomas may be benign (noncancerous) or malignant (cancerous), and either type can be disabling. The bones of the skull prevent the brain from expanding outward as the tumor grows and takes up space. Consequently, healthy brain tissue is compressed, causing symptoms controlled by the area of the brain that is compressed. Because of the pressure either type of astrocytoma (benign or malignant) exerts upon the brain and the resulting symptoms, the distinction between benign (noncancerous) and malignant (cancerous) tumors of the brain is less critical than for tumors occurring anywhere else in the body. Benign astrocytomas are usually more slow growing than the malignant forms. Astrocytomas can occur anywhere in the brain or spinal cord, with the subcortical (beneath the brain covering) white matter (fibrous tissue) of the brain hemispheres being the most common location in adults. The optic nerve, cerebellum and brainstem are the most common locations of benign astrocytomas in children. Symptoms As a benign astrocytoma expands in size, it compresses brain tissue. The growth of the astrocytoma increases pressure in the skull, since both the tumor and the fluid of the brain crowd the surrounding brain tissue. The growth of cysts within the astrocytoma can further increase the size of the tumor. Symptoms of a benign astrocytoma depend on the size, location and rate of growth of the tumor. Onset of symptoms may be sudden, appearing initially in the form of a seizure or cerebral hemorrhage, or the onset may be very gradual and subtle, characterized by slow but progressive mental deterioration. Tumors of the frontal and temporal lobes may grow very large before producing any specific symptoms. Headache is the initial symptom in 50 percent of individuals with benign astrocytoma. The headache usually results from pressure on blood vessels, cranial nerves or pain sensitive tissue. These recurrent headaches often awaken the individual during the night and become much worse in the morning. Seizures are the initial symptom in 20 percent of patients with brain tumors and are more frequently the result of slow growing astrocytomas. Symptoms of a benign astrocytoma may involve mental changes that may not be obvious until the patient's behavior changes substantially. These symptoms may include impersistence in routine tasks, increased irritability, emotional instability, faulty insight, forgetfulness, reduced mental activity, indifference to social practices and loss of initiative or spontaneity. The patient may complain of fatigue, dizziness and lethargy. If the tumor continues to grow, the symptoms may progress to confusion, deteriorated intellectual functioning (dementia) and eventually stupor. These personality changes are often initially confused with symptoms of anxiety or depression. Nausea and vomiting may result from increased pressure on the center in the brain that controls the vomiting reflex (emetic center). Vomiting may occur suddenly and without preceding nausea. Other symptoms may include slowed heartbeat, an inability to control bodily discharge (incontinence), a loss in the ability to recognize the shapes of objects by handling them (astereognosis), paralysis, an inability to coordinate voluntary muscular movements (ataxia), an inability to use or comprehend words (aphasia), rapid involuntary movement of the eyeballs (nystagmus), facial pain or numbness and hearing loss. Causes The exact cause of astrocytomas and other brain tumors is not known. Scientists have suggested that genetic factors, infection, trauma, or suppression of the immune system may contribute to the formation of brain tumors. Affected Population Benign astrocytomas may occur in anyone at any age, but white males between the ages of 40 and 70 are most commonly affected. Related Disorders Symptoms of the following disorders can be similar to those of benign astrocytomas. Comparisons may be useful for a differential diagnosis: Malignant Astrocytomas (or Grade III Astrocytomas) are the most common primary tumors, representing approximately three-fourths of nerve tissue tumors diagnosed yearly in adults in the United States. These rapidly growing tumors most commonly occur in the thick layer of nerve fibers (corpus collasum) that connects the two hemispheres of the brain. Grade III Astrocytomas also occur in the frontal, parietal (top) and temporal (side) lobes of the brain and in the thalamus. (For more information on this disorder, choose "Malignant Astrocytoma" as your search term in the Rare Disease Database.) Motor Neuron Disease is a group of neuromuscular disorders characterized by the progressive degeneration of motor neurons, the nerve cells which control movement and reflex. Symptoms of Motor Neuron Diseases may include muscle weakness, spasms and exaggerated reflexes. (For more information on this disorder, choose "Motor Neuron Disease" as your search term in the Rare Disease Database.) Multiple Sclerosis (MS) is a chronic disease of the brain and spinal cord. MS is characterized by small lesions called plaques that may form randomly throughout the brain and spinal cord. These lesions consist of areas of dissolved myelin, the fatty material that forms a sheath around nerve cells (neurons) and conducts nerve impulses. Large, star-shaped nerve cells (astrocytes) overgrow and harden in the lesions, forming scars in the brain and spinal cord called scleroses. Destruction of the myelin sheath creates a variety of neurological symptoms which may include visual difficulties, impairment of speech, abnormal skin sensations or numbness, walking disturbances and difficulties with bladder and bowel function. (For more information on this disorder, choose "Multiple Sclerosis" as your search term in the Rare Disease Database.) There are many different types of brain tumors, both benign and malignant. Symptoms of each type of brain tumor are related to the place the tumor occurs in the brain and the pressure it exerts on the surrounding tissue. (For more information, type "Brain Tumor" as your search term in the Rare Disease Database.) Therapies: Standard Benign astrocytomas, which may evolve over several years, are detected by CT (computed tomography) scan or by MRI (magnetic resonance imaging). Brain tumors are biopsied even if total removal is not possible. Results of the biopsy will dictate preferred treatment. The major types of therapies used to treat benign astrocytomas after surgery are radiation and chemotherapy. The therapy chosen depends on the type of tumor that is present, its location, and its sensitivity to radiation therapy. Pre-operative treatment is usually aimed at controlling the accumulation of fluid in the brain and the occurrence of seizures. Corticosteroid drugs such as dexamethasone are usually initially prescribed. In cases where steroids do not relieve the accumulation of fluid, a surgically implanted drain (shunt) may be required. Anticonvulsant drugs may be prescribed for individuals who have seizures. Total surgical removal of accessible benign astrocytomas of the cerebellum, lobes and optic nerve is often possible and successful. Accessible tumors are those which can be operated on without causing unacceptably severe damage to other parts of the brain. If surgery is performed, the surgeon will attempt to remove all identifiable parts of the astrocytoma when possible, often using an operating microscope to distinguish 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 objective of removing microscopic tumor infiltrates with a minimal amount of damage to normal tissue. When the astrocytoma involves a crucial part of the brain, partial removal of the growth usually reduces pressure, relieves symptoms and helps control seizures. Full or partial removal of the astrocytoma is sometimes followed by radiation therapy to destroy any remaining tumor cells. With the use of CT (computed tomography) and MRI (magnetic resonance imaging), radiation sometimes may be deferred for several months or years while the patient is scanned at regular intervals. Radiation as primary therapy is occasionally used on low grade (benign) astrocytomas. When radiation is used, it is administered to the tumor and the entire brain, since the astrocytoma can infiltrate surrounding tissue easily. After radiation has reduced the number of tumor cells, chemotherapy is often administered in an attempt to destroy any cells that remain. Chemotherapy may also be given during the course of radiation treatment. The drugs used in chemotherapy are cytotoxins 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 neuro-oncologist who examines the grade of tumor, previous treatment and current health status of the individual with the astrocytoma. Therapies: Investigational One of the new experimental techniques for the treatment of astrocytomas is brachytherapy, also called "interstitial radiation" or "seeding." Via a surgical procedure, radioactive pellets of iodine, iridium or gold isotope are implanted directly into the brain. Brachytherapy is used primarily when a tumor recurs if it 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 neutron, heat (hyperthermia) and light (photoradiation); 3) intraoperative radiation; and 4) hyperfractionation. These are ongoing research projects which are being clinically tested against 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. More research is needed to determine whether these therapies will be successful. A multitude of new drugs and drug combinations are being tested for effectiveness against astrocytomas. 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. During brain surgery, a biodegradable wafer containing a cancer fighting drug (BCNU) is implanted at the site of the tumor. As the wafer dissolves over a period of many weeks, the drug is slowly released. Those interested in this experimental trial should have their physicians contact, Nova Pharmaceutical Corp., 6200 Freeport Centre, Baltimore, MD 21224 or phone 301-522-7000. This disease entry is based upon medical information available through April 1990. 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 Benign Astrocytoma, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 NIH/National Institute of Neurological Disorders & Stroke (NINDS) 9000 Rockville Pike Bethesda, MD 20892 (301) 496-5751 (800) 352-9424 Association for Brain Tumor Research 3725 North Talman Chicago, IL 60618 (312) 286-5571 References CECIL TEXTBOOK OF MEDICINE, 18th ed.: James B. Wyngaarden, M.D. et al., eds; W.B. Saunders Company, 1988. Pp. 2229-2235. INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and Co., 1987. Pp. 2220-2222. THE MERCK MANUAL, Volume 1, 14th Ed.: Robert Berkow, M.D., ed.-in-chief; Merck Sharp & Dohme Laboratories, 1982. Pp. 1406-1410. PROGNOSIS OF BENIGN CEREBELLAR ASTROCYTOMAS IN CHILDREN. J. Szenasy et al.; CHILDS NERV SYST (1983: issue 10 (1)). Pp. 39-47. LOW-GRADE ASTROCYTOMAS: TREATMENT WITH UNCONVENTIONALLY FRACTIONATED EXTERNAL, BEAM STEREOTACTIC RADIATION THERAPY. F. Pozza et al.; RADIOLOGY (May, 1989: issue 171 (2)). Pp. 565-569. BENIGN ASTROCYTIC AND OLIGODENDROCYTIC TUMORS OF THE CEREBRAL HEMISPHERES IN CHILDREN. J. F. Hirsch et al.; J NEUROSURG (April, 1989: issue 70 (4)). Pp. 568-572. LOW-GRADE ASTROCYTOMAS: TREATMENT RESULTS AND PROGNOSTIC VARIABLES. C. A. Medbery 3rd et al.; INT J RADIAT ONCOL BIOL PHYS (October, 1988: issue 15 (4)). Pp. 837-841. LONG-TERM FOLLOW-UP AFTER SURGICAL TREATMENT OF CEREBELLAR ASTROCYTOMAS IN 100 CHILDREN. S. Undjian et al.; CHILDS NERV SYST (April, 1989: issue 5 (2)). Pp. 99-101.