CD-ROM Today 1996 January/CD-ROM Today 1996 January.iso/dp/0420/04207.txt

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$Unique_ID{BRK04207}
$Pretitle{}
$Title{Sarcoma, Ewing's}
$Subject{Sarcoma Ewing's Ewing Tumor Myeloma Endothelial Endothelioma Diffuse
of Bones Osteosarcoma Malignant Lymphoma Chondrosarcoma Osteochondromas}
$Volume{}
$Log{}

Copyright (C) 1990 National Organization for Rare Disorders, Inc.

765:
Sarcoma, Ewing's

** IMPORTANT **
It is possible that the main title of the article (Ewing's Sarcoma) is
not the name you expected.  Please check the SYNONYM listing to find the
alternate names and disorder subdivisions covered by this article.

Synonyms

     Ewing Tumor
     Myeloma, Endothelial
     Endothelioma, Diffuse of Bones

Information on the following diseases can be found in the Related
Disorders section of this report:

     Osteosarcoma
     Malignant Lymphoma
     Chondrosarcoma
     Osteochondromas

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.

Ewing's Sarcoma is a malignant round-cell bone tumor which usually occurs
in the arm or the leg.  It most commonly affects individuals between the ages
of 10 and 20 years.

Symptoms

Ewing's Sarcoma appears as a tumor which can be felt if not seen, and is
usually painful to the touch.  Most of these tumors develop in the bones of
the extremities, but any bone may be affected.  The tumor tends to be
extensive, sometimes involving the entire shaft of a long bone.  It most
commonly occurs in the bone that extends from the hip to the knee (femur) or
from the shoulder to the elbow (humerus).  Sometimes it occurs in the
pelvis.  Pain tends to increase with the duration of the tumor.  There may be
tenderness and swelling in the area, and often intermittent pain that worsens
during the night.  The skin overlying the tumor may be warm and superficial
blood vessels may be prominent.  The affected individual may also develop a
fever and anemia.  If untreated the tumor has a tendency to spread to the
lungs, lymph nodes or the skull.

Causes

Ewing's Sarcoma is usually inherited as an autosomal dominant trait.  Human
traits, including the classic genetic diseases, are the product of the
interaction of two genes, one received from the father and one from the
mother.  In dominant disorders a single copy of the disease gene (received
from either the mother or father) will be expressed "dominating" the other
normal gene and resulting in appearance of the disease.  The risk of
transmitting the disorder from the affected parent to offspring is fifty
percent for each pregnancy regardless of the sex of the resulting child.

Affected Population

Ewing's Sarcoma most commonly occurs in young males between the ages of 10
and 20.  There are approximately 200 different types of cancer, and bone
cancers comprise some of the rarest forms.

Related Disorders

Symptoms of the following disorders can be similar to those of Ewing's
Sarcoma.  Comparisons may be useful for a differential diagnosis:

Osteosarcoma is one of the most common types of primary bone tumor.  It
is highly malignant, and commonly spreads to the lungs.  It is most common in
persons aged 10 to 20 years although it can occur at any age.  Most tumors
are located in the knee region, but can be found in any bone.  A painful mass
is the most usual symptom.

Malignant Lymphoma of the bone is a small round-cell tumor that most
frequently affects adults in their 40's and 50's.  It may arise in any bone.
Pain and swelling are the most common symptoms.  The cancer may spread to
other soft tissue or bone.  When Malignant Lymphoma occurs in the bone
without evidence of disease elsewhere, the outlook is better than for any
other primary malignant bone tumor.  A combination of radiation and
chemotherapy is standard treatment.

Chondrosarcoma is a malignant tumor of the cartilage.  Depending upon the
severity of the tumor, it may grow slowly or rapidly.  Rapid growing tumors
usually spread.  Chondrosarcomas have the ability to seed or implant in
surrounding soft tissues.  Treatment is usually by surgical removal, and care
must be given during surgery to avoid entry into the tumor and spillage of
the contents onto the soft tissues of the wound.

Osteochondroma is the most common benign (noncancerous) bone tumor.  It
occurs most often in persons aged 10 to 20.  An Osteochondroma may occur as a
single or multiple tumor.  A strong familial tendency toward multiple
Osteochondromas may indicate a genetic predisposition to these tumors.

Therapies:  Standard

Treatment of Ewing's Sarcoma consists of various combinations of surgery,
chemotherapy and radiation therapy.  Approximately 50% of patients are cured
with appropriate treatment.

Therapies:  Investigational

This disease entry is based upon medical information available through July
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 Ewing's Sarcoma, please contract:

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

For genetic information and genetic counseling referrals, please contact:

     March of Dimes Birth Defects Foundation
     1275 Mamaroneck Avenue
     White Plains, NY  10605
     [phone]

     Alliance of Genetic Support Groups
     35 Wisconsin Circle, Suite 440
     Chevy Chase, MD  20815
     (800) 336-GENE
     [phone]

References

MENDELIAN INHERITANCE IN MAN, 8th ed.:  Victor A. McKusick; Johns Hopkins
University Press, 1986.  Pp. 242.

THE MERCK MANUAL, Volume 1, 14th Ed.:  Robert Berkow, M.D., ed.-in-chief;
Merck Sharp & Dohme Laboratories, 1982.  Pp. 1301-1303.

IMMUNOHISTOLOGICAL CHARACTERIZATION OF A EWING'S SARCOMA CASE.  S.
Lizard-Nacol et al.; CANCER DETECT PREV (1988:  issue 12 (1-6)).  Pp. 297-302.

LONG-TERM RESULTS IN 144 LOCALIZED EWING'S SARCOMA PATIENTS TREATED WITH
COMBINED THERAPY.  G. Bacci et al.; CANCER (April 15, 1989:  issue 63 (8)).
Pp. 1477-1486.