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Changes in Incidence and Survival of Ewing Sarcoma Patients Over the Past 3 Decades: Surveillance Epidemiology and End Results Data

Esiashvili, Natia MD*; Goodman, Michael MD, MPH; Marcus, Robert B. Jr, MD*

Journal of Pediatric Hematology/Oncology: June 2008 - Volume 30 - Issue 6 - p 425-430
doi: 10.1097/MPH.0b013e31816e22f3
Original Articles

Purpose The US population-based cancer registry Surveillance, Epidemiology, and End Results (SEER) database provides an opportunity to evaluate the incidence and survival rates of Ewing sarcoma (ES) for the past 3 decades. This analysis reflects trends for the diagnosis of localized versus metastatic disease and changes in ES survival in a setting of wide-ranging cancer care institutions across the United States, which is expected to be different from clinical trials published to date.

Materials/Methods Data from the SEER public-access database were reviewed for the diagnosis of ES of the bone among patients of 1 to 19 years of age between 1973 and 2004. Age-adjusted incidence was analyzed for the entire group and for localized and metastatic disease separately over the past 3 decades. Actuarial survival rates were examined for 3 intervals: 1973 to 1982, 1983 to 1992, and 1993 to 2004.

Results The overall incidence of ES seemed to remain unchanged with an average of 2.93 cases/1,000,000 reported annually between 1973 and 2004. The proportion of patients with distant metastasis among all ES cases remained in the 26% to 28% range, whereas the percent of localized cases slightly increased from 57% in 1973 to 1982 to 67% in 1993 to 2004 and the proportion of unstaged cases decreased from 17% to 5%. The 5-year survival of localized disease increased from 44% in the first study decade to 68% in the period after 1993, whereas 5-year survival of metastatic disease increased from 16% to 39%. The corresponding 10-year survival increased from 39% to 63% for localized disease and from 16% to 32% for metastatic ES.

Conclusions The incidence of ES has not increased appreciably over the last 30 years. A marked decrease in the proportion of unstaged cases may be reflective of diagnostic improvement or changes in reporting. There is a clear improvement in survival for both localized and metastatic disease. Poorer outcome of metastatic patients still warrants intensification of therapy, which is currently being tested in several ongoing trials.

*Department of Radiation Oncology, Emory University School of Medicine

Department of Epidemiology Rollins School of Public Health, Emory University, Atlanta, GA

Presented at the Congress of International Society of Pediatric Oncology (SIOP), September 21, 2006, in Geneva.

Reprints: Natia Esiashvili, MD, Department of Radiation Oncology, Emory University School of Medicine, 1365 Clifton Road NE, Atlanta, GA 30322 (e-mail:

Received for publication July 26, 2007; accepted January 23, 2008

© 2008 Lippincott Williams & Wilkins, Inc.