CD-ROM Today 1996 January/CD-ROM Today 1996 January.iso/dp/0377/03772.txt

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$Unique_ID{BRK03772}
$Pretitle{}
$Title{Glioblastoma Multiforme}
$Subject{Glioblastoma Multiforme Giant Cell Glioblastoma Spongioblastoma
Multiforme}
$Volume{}
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Copyright (C) 1986, 1987, 1988, 1989 National Organization for Rare
Disorders, Inc.

281:
Glioblastoma Multiforme

** IMPORTANT **
It is possible the main title of the article (Glioblastoma Multiforme)
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
     Spongioblastoma Multiforme

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.


Glioblastoma multiforme is a highly malignant, rapidly 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

Glioblastoma Multiforme grows very quickly 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.

Glioblastoma Multiforme may occur in any area of the cerebral
hemispheres, but is 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) of the side of the body opposite
the tumor location.

Temporal tumors initially present fewer symptoms, but may include
seizures, motor disturbances such as lack of coordination of body parts, and
language interpretation disturbances.

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 Glioblastoma Multiforme, 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 slightly higher
risk to get Glioblastomas.

Some recent research suggests that glioblastoma may be caused by a rare
virus, but more studies are needed to prove or disprove this theory.

Affected Population

Glioblastoma Multiforme occurs most often in people between the ages of 48
and 60, although it can also affect pre-teenage children.  It affects twice
as many males as females, and whites more frequently than nonwhites.  Blood
type A males appear to be at a higher risk.

Related Disorders

Malignant Astrocytoma is a less malignant form of Glioblastoma.  (For more
information, choose "astrocytoma" as your search term in the Rare Disease
Database.)

Therapies:  Standard

Treatment of this disorder may include surgery, radiation, or chemotherapy.

SURGERY--The treatment of choice for accessible Glioblastoma Multiforme
tumors is surgery.  Accessible tumors are those which can be operated on
without causing unacceptably severe damage to 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 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.

Glioblastomas usually cannot be totally removed because tumor spreading,
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 Glioblastoma 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 Glioblastoma Multiforme.  A
neurooncologist is a physician who has been specially trained and has a
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 Glioblastoma
Multiforme is brachytherapy, also called "interstitial radiation" or
"seeding".  Via a surgical procedure, radioactive pellets such 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 Glioblastoma Multiforme cells.

Immunotherapy aims to stimulate the body's defenses against the tumor.
Using drugs such as interferon, levamisole, interleukin-2, thymosine, and
BCG, it is hoped that the body's own immune system can be stimulated to
fight the tumor.

Clinical trials of the orphan drug sodium monomercaptoundecahydro-orate
(Boralife), as an alternative to conventional photon therapy, are underway.
For additional information, physicians can contact:

     Nuclear Medicine, Inc.
     900 Atlantic Drive, NW
     Atlanta, GA  30332

A multitude of new drugs and drug combinations are being tested for
effectiveness against Glioblastoma.  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 the brain tissue.

A new orphan drug and delivery system is being tested for the treatment
of Glioblastoma and Astrocytoma.  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 [phone].

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 Glioblastoma Multiforme, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     [phone]

     NIH/National Institute of Neurological Disorders & Stroke (NINDS)
     9000 Rockville Pike
     Bethesda, MD  20892
     [phone]
     [phone]

     Association for Brain Tumor Research
     2910 West Montrose Ave.
     Chicago, IL 60618
     [phone]

     American Cancer Society
     1599 Clifton Rd., NE
     Atlanta, GA  30329
     [phone]

     NIH/National Cancer Institute
     9000 Rockville Pike, Bldg. 31, Rm. 1A2A
     Bethesda, MD 20892
     1-800-4-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, [phone] (Neighbor islands call collect)

References

About Glioblastoma Multiforme and Malignant Astrocytoma; D. P. Hesser et.
al., eds.; Association for Brain Tumor Research (1985).