Ewing’s Sarcoma

What is Ewing’s sarcoma?
Ewing’s sarcoma is a type of cancer caused by a genetic abnormality known as a chromosomal translocation–where a piece of one chromosome switches places with a small piece of another chromosome. About 500 new cases of Ewing’s sarcoma are diagnosed each year in the United States. In most of these cases, the cancer has not yet spread. When Ewing’s sarcoma is treated effectively prior to spreading, the five-year survival rate is 60 to 80 percent or better, depending on the size of the tumor and whether or not it can be completely removed.

Who is likely to have Ewing’s sarcoma?
Ewing’s sarcoma usually affects children and young adults and most often develops during puberty, when bones grow rapidly. This cancer is rare among adults. Ewing’s sarcoma is 10 times more likely to occur in Causasian children as in American-American and Asian-American children.

Who is likely to have Ewing’s sarcoma?
Ewing’s sarcoma often develops in the long bones of the arms and legs or in the pelvis or chest. If this cancer spreads, it usually spreads to the lungs, other bones, or bone marrow.

Pain is the most common symptom. Pain may occur at night or with activity at first and then become gradually more severe over time. Also, sometimes swelling, a mass or a lump may form either on the bone or in the tissue around the bone, and this swelling may inhibit joint function or movement. Ewing’s sarcoma can weaken bones and cause them to fracture with little or no stress or trauma. Fever, chills, night sweats, and weight loss are less common but can occur, usually after the cancer spreads beyond its original location.

How does the pathologist make the diagnosis?
The pathologist reviews the results of a personal and family medical history, physical examination, blood tests, and diagnostic tests performed or ordered by the primary care physician. Depending upon the symptoms and other factors, these diagnostic tests may include an x-ray, bone scan, computed tomography (CT) scan, magnetic resonance imaging (MRI) scan, positron emission tomography (PET) scan, or bone marrow evaluation. These tests allow the pathologist to see inside the body to determine the source of pain or the presence of cancer cells.

What else does the pathologist look for?
If a tumor is found, the primary physician may obtain a biopsy specimen–a sample of the tumor’s cells–for the pathologist to examine under a microscope for the presence of cancer cells. The pathologist may also perform a bone marrow aspiration to gather bone marrow cells through a hollow tube for examination, as well as tests looking for chromosome or gene changes in the tumor cells to aid in treatment planning. A physician experienced in the treatment of bone cancer should obtain the biopsy or bone marrow aspiration. To obtain the biopsy sample, the physician uses either a small needle (needle biopsy) or makes a surgical incision (open biopsy) through the skin.

What is meant by the grade and stage of the cancer?
Ewing’s sarcomas are generally regarded as high-grade by the pathologist. However, Ewing’ssarcomas can be classified as intermediate-grade or low-grade. High-grade tumors grow and spread faster than low-grade tumors. The pathologist and primary care doctor also determine the cancer’s stage to plan the best treatment. There is no standard staging system for Ewing’s sarcomas – they are simply classified as either localized (not without spread) or metastatic.

How do doctors determine what treatment will be necessary?
The recommended treatment will depend on the grade and stage of the tumor, its size and location, and the patient’s age and general health. The pathologist consults with the primary care physician or specialist. Together, using their combined experience and knowledge, they determine treatment options most appropriate for the patient’s condition. It’s important to learn as much as possible about the treatment options and make the decision that’s right for you.

What kinds of treatments are available for Ewing’s sarcoma?
Treatment for Ewing’s sarcoma usually includes chemotherapy followed by surgery and radiation therapy.

Chemotherapy kills cancer cells through the use of anti-cancer drugs. Most patients with Ewing’s sarcoma receive a combination of these drugs. Physicians use chemotherapy before surgery to reduce the size of the bone tumor, as well as after surgery to kill cells remaining after surgery. Most Ewing’s sarcoma patients receive chemotherapy for several months.

The usual surgical treatment for Ewing’s sarcoma involves the surgeon removing the entire tumor along with a margin of cancer-free tissue surrounding the tumor. In most cases, surgeons are able to avoid the removal of an entire limb. In some cases, reconstructive surgery or limb-salvaging surgery is required for the patient to continue to have limb function. Depending on the amount of bone removed, the surgeon may use bone cement, a bone graft, or metal implants to maintain limb function.

Radiation therapy uses pinpointed, high-energy beams to kill cancer cells and can be used before or after surgery or for patients who refuse or cannot have surgery. Radiation therapy can reduce the risk of cancer spreading to tissue surrounding the tumor and can relieve pain and other symptoms. Two types of radiation therapy can be used–external radiation, during which radiation comes from a large machine, or internal radiation, during which radiation is received through thin tubes (also called implants) inserted in or near the cancerous cells.

In addition, people with Ewing’s sarcoma may receive other treatments to control pain and other symptoms, to relieve side effects or to ease emotional problems. Many patients also require rehabilitation to adapt to reconstructed or salvaged limbs or new prosthetic devices.

Ewing’s sarcoma sometimes spreads or comes back, and people with bone cancer also are more likely to develop another type of cancer. For these reasons, follow-up treatment is necessary. The type of follow-up treatment depends on the grade and stage of the cancer. Regular blood tests and x-rays or other imaging tests are usually necessary, and patients must be vigilant about reporting any unusual symptoms.

Clinical trials of new treatments for chondrosarcoma may be found at www.cancer.gov/clinicaltrials. These treatments are highly experimental in nature but may be an option for advanced cancers. For more information about clinical trials, talk to your doctor or call the National Cancer Institute’s Cancer Information Service at 1–800–4–CANCER.

For more information, go to www.cancer.gov (National Cancer Institute) or http://www.sarctrials.org/upload/bonecancersarcpatientguidelines_87081.pdf (Sarcoma Alliance for Research through Collaboration). Type the keywords Ewing’s sarcoma into the search box.

What kinds of questions should I ask my doctors?
Ask any question you want. There are no questions you should be reluctant to ask. Here are a few to consider:

  • Please describe the type of cancer I have and what treatment options are available.
  • What is the grade and stage of my cancer?
  • What are the chances for full remission?
  • What treatment options do you recommend? Why do you believe these are the best treatments?
  • What are the pros and cons of these treatment options?
  • What are the side effects?
  • Is your medical team experienced in treating the type of cancer I have?
  • Can you provide me with information about the physicians and others on the medical team?
  • If I want a second opinion, could you provide me with the names of physicians and/or institutions that you would recommend?

DEFINITION OF TERMS


Malignant: Cancerous.

Metastatic: Spreading beyond the original tumor location to other parts of the body.

Pathologist: A physician who examines tissues and fluids to diagnose disease in order to assist in making treatment decisions.

Sarcoma: A cancer–usually malignant–rising from bone, muscle or other connective tissue.

 

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Ewing’s Sarcoma

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This information was developed by the College of American Pathologists’ Public Affairs Committee in conjunction with the College’s Cancer Committee and Council on Scientific Affairs. The College is providing this information to help you better understand your health. Ultimately any decisions you make about your health, however, should be between you and your doctor.

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