Ewing Sarcoma Treatment (PDQ®)–Health Professional Version

General Information About Ewing Sarcoma

Dramatic improvements in survival have been achieved for children and adolescents with cancer.[1] Between 1975 and 2010, childhood cancer mortality decreased by more than 50%.[1] For Ewing sarcoma, the 5-year survival rate has increased over the same time from 59% to 78% for children younger than 15 years and from 20% to 60% for adolescents aged 15 to 19 years.[1]

Studies using immunohistochemical markers,[2] cytogenetics,[3,4] molecular genetics, and tissue culture [5] indicate that Ewing sarcoma is derived from a primordial bone marrow–derived mesenchymal stem cell.[6,7] Older terms such as peripheral primitive neuroectodermal tumor, Askin tumor (Ewing sarcoma of chest wall), and extraosseous Ewing sarcoma (often combined in the term Ewing sarcoma family of tumors) refer to this same tumor.

Incidence

The incidence of Ewing sarcoma has remained unchanged for 30 years.[8] The incidence for all ages is one case per 1 million people in the United States. In patients aged 10 to 19 years, the incidence is between nine and ten cases per 1 million people. The same analysis suggests that the incidence of Ewing sarcoma in the United States is nine times greater in whites than in African Americans, with an intermediate incidence in Asians.[9,10]

The relative paucity of Ewing sarcoma in people of African or Asian descent may be explained, in part, by a specific polymorphism in the EGR2 gene.[11]

The median age of patients with Ewing sarcoma is 15 years, and more than 50% of patients are adolescents. Well-characterized cases of Ewing sarcoma in neonates and infants have been described.[12,13] Based on data from 1,426 patients entered on European Intergroup Cooperative Ewing Sarcoma Studies, 59% of patients are male and 41% are female.[14]

Clinical Presentation

Primary sites of bone disease include the following:

Lower extremity (41%).
Pelvis (26%).
Chest wall (16%).
Upper extremity (9%).
Spine (6%).
Hand and foot (3%).[15]
Skull (2%).

For extraosseous primary tumors, the most common primary sites of disease include the following:[16,17]

Trunk (32%).
Extremity (26%).
Head and neck (18%).
Retroperitoneum (16%).
Other sites (9%).

The median time from first symptom to diagnosis of Ewing sarcoma is often long, with a median interval reported from 2 to 5 months. Longer times are associated with older age and pelvic primary sites. Time from first symptom to diagnosis has not been associated with metastasis, surgical outcome, or survival.[18] Approximately 25% of patients with Ewing sarcoma have metastatic disease at the time of diagnosis.[8]

The Surveillance, Epidemiology, and End Results (SEER) database was used to compare patients younger than 40 years with Ewing sarcoma who presented with skeletal and extraosseous primary sites (refer to Table 1).[19] Patients with extraosseous Ewing sarcoma were more likely to be older, female, nonwhite, and have axial primary sites, and were less likely to have pelvic primary sites than were patients with skeletal Ewing sarcoma.