rarediseases.

org/rare-diseases/anaplastic-astrocytoma

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.

Synonyms of Anaplastic Astrocytoma

astrocytoma, grade III
malignant astrocytoma, grade III

General Discussion
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.

Signs & Symptoms

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

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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.

Causes
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.

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Affected Populations
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.

Related Disorders
Symptoms of the following disorders can be similar to those of anaplastic astrocytoma.
Comparisons may be useful for a differential diagnosis.

Other brain tumors must be distinguished from anaplastic astrocytomas. Such tumors
include metastatic tumors, lymphomas, hemangioblastoma,s craniopharyngiomas,
teratomas, ependymomas, and medulloblastomas. Additional conditions that may resemble
an anaplastic astrocytoma include inflammation of the membranes (meninges) that
surround the brain and spinal cord (meningitis) and pseudotumor cerebri (benign
intracranial hypertension). (For more information on these disorders, choose the specific
disorder name as your search term in the Rare Disease Database.)

Diagnosis
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.

Standard Therapies
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Treatment

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
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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.

Investigational Therapies
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: prpl@cc.nih.gov

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
212-305-7346

NORD Member Organizations

American Brain Tumor Association
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 8550 W. Bryn Mawr Avenue, Suite 550
Chicago, IL 60631 USA
Phone: (773) 577-8750
Toll-free: (800) 886-2282
Email: info@abta.org
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National Brain Tumor Society
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Phone: (617) 924-9997
Toll-free: (800) 770-8287
Email: info@braintumor.org
Website: http://www.braintumor.org

Other Organizations
American Cancer Society, Inc.
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Phone: (404) 320-3333
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American Childhood Cancer Organization
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Kensington, MD 20895
Phone: (301) 962-3520
Toll-free: (800) 366-2223
Email: staff@acco.org
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Brain Tumor Foundation for Children, Inc.
6065 Roswell Road Suite 505
Atlanta, GA 30328-4015 USA
Phone: (404) 252-4107
Email: info@braintumorkids.org
Website: http://www.braintumorkids.org
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 Toll-free: (888) 793-9355
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Cancer.Net
American Society of Clinical Oncology
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Toll-free: (888) 651-3038
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Ontario, M4T 1L9 Canada
Phone: (416) 489-6440
Toll-free: (800) 363-1062
Email: info@childhoodcancer.ca
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Children’s Brain Tumor Foundation
274 Madison Avenue, Suite 1004
New York, NY 10016 United States
Phone: (212) 448-1595
Toll-free: (866) 228-4673
Email: info@cbtf.org
Website: http://www.cbtf.org
Friends of Cancer Research
1800 M Street NW
Suite 1050 South
Washington, DC 20036
Phone: (202) 944-6700
Email: info@focr.org
Website: http://www.focr.org
Harvard Brain Tissue Resource Center
McLean Hospital
115 Mill Street
Belmont, MA 2178
Phone: (617) 855-2400
Toll-free: (800) 272-4622
Email: hbtrc@mclean.harvard.edu
Website: http://www.brainbank.mclean.org
Livestrong Foundation

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 2201 E. Sixth Street
Austin, TX 78702
Phone: (512) 236-8820
Toll-free: (877) 236-8820
Email: media@livestrong.org
Website: http://www.livestrong.org
NIH/National Institute of Neurological Disorders and Stroke
P.O. Box 5801
Bethesda, MD 20824
Phone: (301) 496-5751
Toll-free: (800) 352-9424
Website: http://www.ninds.nih.gov/
Pediatric Brain Tumor Foundation
302 Ridgefield Court
Asheville, NC 28806
Phone: (828) 665-6891
Toll-free: (800) 253-6530
Email: info@curethekids.org
Website: http://www.pbtfus.org
Rare Cancer Alliance
1649 North Pacana Way
Green Valley, AZ 85614 USA
Website: http://www.rare-cancer.org

References
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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.
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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.

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Radiother Oncol. 1993;27:181-92.

INTERNET
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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