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What is the Ewing's Sarcoma Family of Tumors?Tumors in the Ewing's family of sarcomas are made of primitive cells, which are cells that haven't yet decided what type of cell they are. They look blue to a pathologist due to the staining that is used in the process of identifying the cancer so the cells are referred to as "small round blue cells" as shown in this pathologist's slide. The Ewing's family of sarcomas includes:
Each of these cancer is frequently referred to "Ewing's sarcoma" in the medical and lay literature. They are a subtype of sarcomas which, as noted above, are tumors in the connective tissues. Some useful information about Ewing's sarcoma can be found at the following links:
Detailed Descriptions of the Disease Dr. R. Lor Randall, Director of Sarcoma Services at the Huntsman Cancer Institute and Primary Children’s Medical Center at the University of Utah, has written an in-depth article about this disease, Ewing’s Sarcoma Family of Tumors (ESFT), for the Electronic Sarcoma Update Newsletter (ESUN). His article gives an extensive set of references to articles dealing with ESFT.
A complementary article, Ewing Sarcoma and Primitive Neuroectodermal Tumors by Dr. Toretsky, appears on the E-Medicine website.
Drs. Daliani and Patel, in their article, Soft-Tissue and Bone Sarcomas, state that Ewing's sarcoma "is considered a systemic disease; even when a tumor is apparently localized, approximately 90% of the cases include occult metastatic disease".
Where do Ewing's Sarcoma Tumors Occur? The Detailed Guide: Ewing's Family of Tumors states, "The majority of these tumors occur in the trunk, and the most common site is the pelvis. About one third of the bone tumors occurs in the legs, mainly in the middle of the long bones. In contrast, osteosarcoma usually occurs at the ends of the long bones, especially around the knees. The next most common sites are the ribs and spine. The extraosseous tumors can occur almost anywhere."
How is Ewing's Sarcoma Treated?Dr. Randall, in the article cited above, states that, "It is critical that a patient diagnosed with ESFT is treated at a center very familiar with this disease". He goes on to state, "Advances in chemotherapy (CTx) have significantly improved survival. Surgical removal of the primary tumor generally occurs after a course of initial chemotherapy. Chemotherapy is started first to attack any potential tumor cells that have broken off from the main tumor (metastasized) but have not yet been detected by the staging studies."
Currently, the treatment of Ewing's sarcoma (soft tissue tumors and bone tumors) are the same. Based on the results of a a number of clinical trials the first line treatment is quite standardized and consists of:
What is the Prognosis for Patients with Ewing's Sarcoma?An important characterization of the cancer is whether it is local or if it has spread (i.e., metastasized). If there are no metastases at diagnosis, the 5-year prognosis is 70% for children and 50% for adults. If there are metastases, the prognosis is much lower (10-25%). The prognosis is not good for patients that develop metastases while in treatment; see our Prognosis page.
How is Ewing's Sarcoma Diagnosed?If Ewing's sarcoma metastasizes, it normally spreads to the lungs, bones and bone marrow. Chest CT scans are performed to check the lungs. If the primary tumor was in the bone, bone marrow biopsies are performed. Bone scans check for tumors in the bones. Other scans, such as an MRI scan, angiogram, and a PET scans are also used, from time to time, for diagnostic purposes. A biopsy will be required in order for a pathologist to indicate the specific subtype of sarcoma. Either a fine needle aspiration, core need biopsy or a surgical biopsy will be performed. The tissue should be classified by a pathologist who has substantial background in diagnosing sarcomas. It is quite common to seek a second opinion from a pathologist at a cancer center that has expertise in dealing with sarcoma.
Who develops Ewing's Sarcomas?There are over 50 subtypes of sarcoma. The American Cancer Society's Key Statistics About Sarcoma states that the most common types of soft tissue sarcoma are: MFH or Malignant Fibrous Histiocytoma (28%), liposarcoma (15%), LMS or leiomyosarcoma (12%), synovial sarcoma (10%), malignant peripheral nerve sheath tumors (6%), and rhabdomyosarcoma (5%). All of the other types of soft tissue sarcoma occur at percentages of 3% or less. The ACS' Key Statistics for Bone Cancer states that the most common types of bone cancer are osteosarcoma (35%), chondrosarcoma (26%), Ewing's bone sarcoma (16%), chordoma (8%), and malignant fibrous histiocytoma/fibrosarcoma (6%). Other rare bone cancers account for the remaining 9%.
About 75% of the cases of Ewing's sarcoma involve young patients age 5 to 20 years. Since Ewing's sarcoma has a higher incidence in children than in adults, it is considered a "pediatric cancer". The median patient age is 15 years old. There are approximately 200 new cases diagnosed in children and adolescents in the US per year (Improved Survival for Those With Ewing's Sarcoma, National Cancer Institute), and 20 diagnosed in adults (European Society for Medical Oncology).
Our Ewing's Sarcoma Resources You can find out more about the location, prognosis, refractory and recurrent disease, treatments and tests associated with Ewing's sarcoma by clicking on the appropriate button on the left.
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