NORD gratefully acknowledges Jeffrey N. Bruce, MD, Professor of Neurological Surgery, Vice Chairman, Neurosurgery, Columbia University College of Physicians and Surgeons, for assistance in the preparation of this report.
Anaplastic astrocytoma is a rare malignant brain tumor. Astrocytomas are tumors that develop from certain star-shaped brain cells called astrocytes. Astrocytes and similar cells form tissue that surrounds and protects other nerve cells found within the brain and spinal cord. Collectively, these cells are known as glial cells and the tissue they form is known as glial tissue. Tumors that arise from glial tissue, including astrocytomas, are collectively referred to as gliomas. The symptoms of anaplastic astrocytomas vary depending upon the specific location and size of the tumor. The specific cause of this tumor is unknown.
Astrocytomas are classified according to a grading system developed by the World Health Organization (WHO). Astrocytomas come in four grades based upon how fast the cells are reproducing and that likelihood that they will spread (infiltrate) nearby tissue. Grades I or II astrocytomas are nonmalignant and may be referred to as low-grade. Grades III and IV astrocytomas are malignant and may be referred to as high-grade astrocytomas. Anaplastic astrocytomas are grade III astrocytomas. Grade IV astrocytomas are known as glioblastoma multiforme. Lower grade astrocytomas can change into higher grade astrocytomas over time.
The symptoms of anaplastic astrocytoma vary depending upon the exact location and size of the tumor. Most symptoms result from increased pressure within the brain. An anaplastic astrocytoma usually develops slowly over time, but may develop rapidly.
Increased pressure within the brain may be caused by the tumor itself or by blockage of the fluid-filled spaces in the brain called ventricles, which results in the abnormal accumulation of cerebrospinal fluid (CSF) in the brain. Symptoms commonly associated with anaplastic astrocytomas include headaches, lethargy or drowsiness, vomiting, and changes in personality or mental status. In some cases, seizures, vision problems, weakness of the arms and legs resulting in coordination difficulties may also occur.
More specific symptoms relate to the area of brain where the tumor is located. Anaplastic astrocytomas may develop in any area of the central nervous system, although there is a strong preference for the large rounded portion of the brain (cerebrum) that occupies most of the skull. The cerebrum is divided into two halves known as the cerebral hemispheres. Anaplastic astrocytomas may develop in the frontal, temporal, parietal and occipital lobes of the cerebrum.
A tumor in the frontal lobe may cause memory problems, changes in personality and mood, and paralysis (hemiplegia) on the side of the body opposite of the tumor. Tumors in the temporal lobe may cause seizures, memory problems, and problems with coordination and speech. Tumors in the parietal lobe may cause difficulties with communication through writing (agraphia), problems with fine motor skills, or sensory abnormalities such as tingling or burning sensations (paresthesias). Tumors in the occipital lobe can cause visual loss.
Other common sites for anaplastic astrocytomas include the part of the brain that contains the thalamus and hypothalamus (diencephalon), the lower area of brain near the back of the neck that controls movement and balance (cerebellum), and the spinal cord. Tumors in the diencephalon region may cause headaches, fatigue, weakness of the arms and legs, vision problems, and hormonal imbalances. Tumors in the cerebellum may cause headaches, changes in personality or behavior, and balance problems. Tumors of spinal cord may cause back pain, sensory abnormalities such as tingling or burning sensations (paresthesias), weakness, and gait disturbances.
The exact cause of anaplastic astrocytomas is unknown. Researchers speculate that genetic and immunologic abnormalities, environmental factors (e.g., exposure to ultraviolet rays, certain chemicals, ionizing radiation), diet, stress, and/or other factors may play contributing roles in causing specific types of cancer. Investigators are conducting ongoing basic research to learn more about the many factors that may result in cancer.
Astrocytomas occur with greater frequency in certain genetic disorders including neurofibromatosis type I, Li-Fraumeni syndrome, and tuberous sclerosis. Except in these rare disorders, the vast majority of astrocytomas are not passed on to offspring with greater frequency. Researchers believe that some individuals may have a genetic predisposition to developing an astrocytoma. A person who is genetically predisposed to a disorder carries a gene (or genes) for the disease, but it may not be expressed unless it is triggered or “activated” under certain circumstances, such as due to particular environmental factors.
Anaplastic astrocytomas affect males slightly more often than females. The exact incidence of these tumors is unknown. Anaplastic astrocytoma and glioblastoma multiforme are estimated to affect 5-8 people per 100,000 in the general population. Anaplastic astrocytomas are more common in adults than children. In adults, anaplastic astrocytomas usually develop between 30 and 50 years of age. Astrocytomas and a related tumor (oligodendroglioma) are the most common primary brain tumors in adults.
In children, anaplastic astrocytomas usually develop between 5 and 9 years of age. Malignant astrocytomas (i.e., anaplastic astrocytoma and glioblastoma multiforme) account for approximately 10 percent of childhood central nervous stem tumors. Astrocytomas as a whole account for more than half of all primary childhood tumors of the central nervous system. Most astrocytomas (approximately 80 percent) in children are low grade.
A diagnosis of anaplastic astrocytoma is made based upon a thorough clinical evaluation, a detailed patient history, and a variety of imaging techniques including computerized tomography (CT) scanning and magnetic resonance imaging (MRI). During CT scanning, a computer and x-rays are used to create a film showing cross-sectional images of certain tissue structures. An MRI uses a magnetic field and radio waves to produce cross-sectional images of particular organs and bodily tissues. Such imaging techniques may also be used to may be used to help evaluate the size, placement, and extension of the tumor and to serve as an aid for future surgical procedures.
Surgical removal and microscopic evaluation (biopsy) of tissue from a tumor may confirm a diagnosis.
The therapeutic management of individuals with an anaplastic astrocytoma may require the coordinated efforts of a team of medical professionals, such as physicians who specialize in the diagnosis and treatment of cancer (medical oncologists), specialists in the use of radiation to treat cancer (radiation oncologists), surgeons, neurologists, oncology nurses, and other health care specialists.
Specific therapeutic procedures and interventions may vary, depending upon numerous factors, such as primary tumor location, extent of the primary tumor (stage), and degree of malignancy (grade); whether the tumor has spread to lymph nodes or distant sites (which rarely occurs with astrocytomas); an individual’s age and general health; and/or other elements. Decisions concerning the use of particular interventions should be made by physicians and other members of the health care team in careful consultation with the patient, based upon the specifics of the case; a thorough discussion of the potential benefits and risks; patient preference; and other appropriate factors.
The three main forms of treatment for anaplastic astrocytoma are surgery, radiation and chemotherapy. These treatments may be used alone or in combination with one another. The initial treatment in most cases is surgical excision and removal of as much as the tumor as possible (resection). Sometimes, only a portion of the tumor can be safely removed because malignant cells may have spread into surrounding brain tissue. Because surgery often cannot completely remove a tumor, radiation therapy and chemotherapy are usually used following surgery to continue treatment.
Postoperative radiation to help treat known or possible residual disease is frequently used in anaplastic astrocytomas. If initial surgery is not an option due to the specific location and/or progression of the malignancy, therapy may include radiation alone. Radiation therapy preferentially destroys or injures rapidly dividing cells, primarily cancerous cells. However, some healthy cells (e.g., hair follicles, bone marrow, etc.) may also be damaged, leading to certain side effects. Thus, during such therapy, the radiation is passed through diseased tissue in carefully calculated dosages to destroy cancer cells while minimizing exposure and damage to normal cells. Radiation therapy works to destroy cancer cells by depositing energy that damages their genetic material, preventing or slowing their growth and replication.
Therapy with certain anticancer drugs (chemotherapy) may also be used to treat individuals with anaplastic astrocytoma. Only one chemotherapeutic agent has been approved for adults with anaplastic astrocytoma. No agents are approved for use in children. Most chemotherapeutic agents have demonstrated only limited effectiveness for treating individuals with anaplastic astrocytoma.
The Food and Drug Administration (FDA) has approved temozolomide (Temodar) for the treatment of adults with anaplastic astrocytoma that has not responded to other forms of therapy (refractory anaplastic astrocytoma). Temodar is manufactured by the Schering-Plough Corporation.
A variety of new therapies are under investigation as potential treatment for individuals with anaplastic astrocytoma. Such therapies include several classes of drugs including protein kinase inhibitors, biological response modifiers, and angiogenesis inhibitors. High dose chemotherapy with auto-bone marrow transplantation is also being investigated for anaplastic astrocytoma. Convection enhanced delivery is a novel experimental method to deliver high levels of chemotherapy directly into the tumor and surrounding brain tissue. Immunotherapy with tumor vaccines is in the early stages of testing.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Toll-free: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]
For information about clinical trials sponsored by private sources, in the main, contact: www.centerwatch.com
For more information about clinical trials conducted in Europe, contact: https://www.clinicaltrialsregister.eu/
Contact for additional information about anaplastic astrocytoma:
Jeffrey N. Bruce, M.D.
Professor of Neurological Surgery
Vice Chairman, Neurosurgery
Columbia University College of Physicians and Surgeons
Neurological Institute, Room 434
710 West 168th St.
New York, N.Y. 10032
Omuro A, DeAngelis LM. Glioblastoma and other malignant gliomas: a clinical review. JAMA. 2013 Nov 6;310(17):1842-50.
Van Meir EG, Hadjipanayis CG, Norden AD, Shu HK, Wen PY, Olson JJ. Exciting new advances in neuro-oncology: the avenue to a cure for malignant glioma. CA Cancer J Clin. 2010 May-Jun;60(3):166-93.
Koukourakis GV, Kouloulias V, Zacharias G, et al. Temozolomide with radiation therapy in high grade brain gliomas: pharmaceutical considerations and efficacy; a review article. Molecules. 2009;14:1561-1577.
Wick W, Platten M, Weller M. New (alternative) temozolomide regimens for the treatment of glioma. Neuro Oncol. 2009;11:69-79.
Belda-Iniesta C, de Castro Carpeno J, Casado Saenz E, et al., Molecular biology of malignant gliomas. Clin Transl Oncol. 2006;8:635-41.
Mathieu D, Fortin D. The role of chemotherapy in the treatment of malignant astrocytomas. Can J Neurol Sci. 2006;33:127-40.
Reardon DA, Rich JN, Friedman HS, Bigner DD. Recent advances in the treatment of malignant astrocytoma. J Clin Oncol. 2006;24:1253-65.
Chang SM, Parney IF, Huang W, et al. Patterns of care for adults with newly diagnosed malignant glioma. JAMA. 2005 Feb 2;293(5):557-64.
Gonzalez J, Gilbert MR. Treatment of astrocytomas. Curr Opin Neurol. 2005;18:632-8.
See SJ, Gilbert MR. Anaplastic astrocytoma: diagnosis, prognosis, and management. Semin Oncol. 2004;31:618-34.
Jennings MT, Ivengar S. Pharmacotherapy of malignant astrocytomas of children and adults: current strategies and future trends. CNS Drugs. 2001;15:719-43.
Mornex F, Nayel H, Taillander L. Radiation therapy for malignant astrocytomas in adults. Radiother Oncol. 1993;27:181-92.
Kennedy B, Bruce JN. Astrocytoma. Medscape. Last Update August 22, 2016. Available at: http://emedicine.medscape.com/article/283453-overview Accessed December 13, 2016.
MacDonald T, Packer RJ. Astrocytoma. Medscape. Last Update November 25, 2014. Available at: http://www.emedicine.com/ped/topic154.htm Accessed December 13, 2016.
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