$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 (203) 746-6518 American Cancer Society 1599 Clifton Rd., NE Atlanta, GA 30329 (404) 320-3333 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, (808) 524-1234 (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 (914) 428-7100 Alliance of Genetic Support Groups 35 Wisconsin Circle, Suite 440 Chevy Chase, MD 20815 (800) 336-GENE (301) 652-5553 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.