Part 1 — Introduction and Symptoms

What is Ewing's Sarcoma?

Ewing's sarcoma is the most common type of sarcoma (cancer of bone and soft tissues) in children under the age of 10. Most children diagnosed with this are between the ages of 5 and 25. The cancer is named after one of the first pathologists to describe it.

Ewing's sarcoma usually starts within bone, but occasionally arises in soft tissues. There is no definitive evidence that Ewing's produces a specific type of tissue, in contrast to osteosarcoma, which produces bone. Nevertheless, there appears to be some relationship to immature or primitive nerve cells. The tumor is composed of small round blue cells. The cells of Ewing's have a typical abnormality in the DNA or genes, but there is no evidence that the susceptibility to this tumor is inherited.

Incidence and Prevalence

The peak incidence of Ewing's sarcoma occurs among children between the ages of 10 and 20, and it is infrequent in children under 5 or adults over 30. Prior to adolescence, the number of males and females affected are equal. After adolescence, the likelihood of new diagnoses is slightly higher for males than females. Ewing's tumors account for 4 percent of all childhood and adolescent malignancies. Ewing's can occur in any bone in the body. However, the most common sites are the pelvis, thigh, lower leg, upper arm, and rib. Ewing's sarcoma can also arise in soft tissue, or an "extra-skeletal" site. In the general population, the annual incidence of Ewing's sarcoma from birth to age 20 is 2.9 cases per million. Approximately 10 percent of patients with Ewing's sarcoma are between 20 and 30 years. Cases occurring later in life are infrequent.

Symptoms

Looking over X-rays.

Patients with Ewing's sarcoma usually go to their doctor because of pain. They might also feel a mass or lump, and have a fever. The pain is usually out of proportion, in both severity and duration, to any specific trauma or injury. These children will often have pain at night. The cancer is frequently found at the ends of bones (near the joints) or in the shaft (middle) of bones.

The diagnosis of Ewing's sarcoma is made by a biopsy, a small sample of tissue that is taken and examined under a microscope for the presence of cancer. Ewing's sarcoma can spread through the blood stream to the lungs, other bones, or bone marrow. At the initial diagnosis, about 25 percent of patients will have cancer that has spread, called metastases (meh-TAS'-tuh-seez). Metastases are found by doing a computed tomography (CAT or CT) scan of the lungs; a bone scan to examine the rest of the skeleton; and an aspirate of the bone marrow of the pelvis, usually with a needle.


For more information, see:

Part 2 — Treatment, Side Effects, Restrictions, and Implications for School

Curesearch

National Library of Medicine/National Institutes of Health

Contributed by:

Kim Templeton, MD
Professor
Department of Orthopedic Surgery
University of Kansas Medical Center

Kathy Davis, MSEd, PhD
Associate Professor
Project Director, Connected Kansas Kids
Director, KU Kids Healing Place
University of Kansas Medical Center