Ewing Sarcoma Family of Tumors

Ewing Sarcoma at a Glance

• Ewing sarcoma family of tumors is a rare type of primary bone cancer.
• The median age of patients is 15 years, and more than 50% of patients are adolescents.
• The incidence is nine times greater in U.S. Caucasians than African Americans.
• Possible Signs of Ewing sarcoma family of tumors include pain and/or swelling, most commonly in the arms, legs, chest, back, or pelvis (area between the hips); a lump (which may feel warm) in the arms, legs, chest, or pelvis; fever for no known reason, a bone that breaks for no known reason.

What Is Ewing sarcoma family of tumors?
Ewing sarcoma family of tumors is a group of cancers of the bone and of soft tissue that form from a certain kind of cell in bone or soft tissue. This family of tumors includes the following:

• Ewing tumor of bone-- This type of tumor is found in the bones of the legs, arms, chest, trunk, back, or head. There are three types of Ewing tumor of bone:
• Classic Ewing sarcoma.
• Primitive neuroectodermal tumor (PNET).
• Askin tumor (PNET of the chest wall).
• Extraosseous Ewing sarcoma (tumor growing in tissue other than bone)-- This type of soft tissue tumor is found in the trunk, arms, legs, head, and neck.

Incidence

The incidence of Ewing sarcoma family of tumors (ESFTs) is approximately three per 1,000,000 per year and remained unchanged for 30 years. Data from the Surveillance, Epidemiology, and End Results (SEER) registries reports an overall incidence of ESFT of one per 1,000,000 in the U.S. population. The incidence in patients aged 10 to 19 years is between nine and ten per 1,000,000. The same analysis suggests that the incidence of Ewing sarcoma is nine times greater in U.S. Caucasians than African Americans. The median age of patients with ESFT is 15 years, and more than 50% of patients are adolescents. Well-characterized cases of ESFT in neonates and infants have been described. Based on data from 1,426 patients entered on European Intergroup Cooperative Ewing Sarcoma Studies (EI-CESS), 59% of patients are male and 41% are female.

Signs and Symptoms

Possible signs of Ewing sarcoma family of tumors include:

• Pain and/or swelling, most commonly in the arms, legs, chest, back, or pelvis (area between the hips).
• A lump (which may feel warm) in the arms, legs, chest, or pelvis.
• Fever for no known reason.
• A bone that breaks for no known reason.