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Abstract
Background. Ewing’s sarcoma family of tumors (ESFTs) are rare bone and soft tissue tumors characterized by specific genetic alterations. Our aim was to carry out a nationwide analysis of ESFT, to survey the treatments used and to report the five-year disease specific and event-free survival rates (EFS and DSS). Material and methods. The study data was gathered from the Finnish National Cancer Registry and all five University Hospitals and consisted of 76 bone and soft tissue ESFT patients diagnosed during 1990–2009. Their medical records were reviewed and data on their disease, treatments, complications and outcome were analyzed. Results. The five-year EFS and DSS of patients with localized disease at diagnosis (n = 57) were 70% and 60%, respectively. Factors contributing to DSS and EFS were the axial vs. peripheral site of primary tumor and adequate surgical resection of the primary tumor. DSS was also affected by patient’s age at diagnosis and the treatment employed. The five-year DSS of patients with metastatic disease at diagnosis (n = 19) was 33% and both preoperative and high dose chemotherapy were associated with improved survival. Conclusion. Population-based studies including both bone and soft tissue ESFTs are few. In this nationwide, population-based study on Finnish bone and soft tissue ESFT patients, we find their treatment successful and results comparable to those previously published. Absence of metastases, young age at diagnosis and a peripheral primary tumor site were associated with a better prognosis. It seems that surgical resection of the primary tumor should be performed whenever adequate resection margins can be achieved. The role of high dose chemotherapy merits further studies in this setting.
Acknowledgements
We thank Mikko Perkkiö, Willy Serlo and Jaana Vettenranta for their aid in collecting the data.
Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
We gratefully acknowledge funding from the Nona and Kullervo Väre Foundation, Finnish Pediatric Research Foundation, Medtronic, Baxter and EVO funds. IH has been working as a consultant for Medtronic and MT for MSD, JS has received financial support for conference expenses from Medtronic.