Abstract
BackgroundExtraskeletal Ewings sarcoma are rare tumors for which there is no consensus on optimal management.MethodsA retrospective review of the clinical features, treatment and outcome of patients with extraskeletal Ewings sarcoma who reported to a single institution between January 1992 – December 2003 is reported.ResultsA total of 19 patients with extraskeletal Ewings sarcoma were identified. Of these, 4 patients had metastatic disease at presentation and 15 patients with non-metastatic disease received combined modality treatment with primary combination chemotherapy followed by local treatment with radiotherapy or surgery. Disease free survival and overall survival for patients with non metastatic disease after combined modality treatment were 60% and 30% respectively. The significant predictors for prolonged disease free survival and overall survival were high haemoglobin(p = 0.002), low lactate dehydrogenase (p = 0.028), chemotherapy with Vincristine, Adriamycin, Cyclophosphamide, Ifosfamide and Etoposide regime (p = 0.008) and complete response to chemotherapy (p = 0.001).ConclusionAggressive combination chemotherapy followed by complete surgery or radiotherapy to a dose of more than 50 Gy is essential to confer optimal outcome for patients with extraskeletal ewings sarcoma.
Highlights
Extraskeletal Ewings sarcoma are rare tumors for which there is no consensus on optimal management
extraskeletal Ewings sarcoma (ESES) patients are offered combined modality treatment as for Ewing's sarcoma (ES) of the same site and stage, though some studies have suggested that extraosseous primaries may have a poorer prognosis[1]
None of the patients who had a complete response to chemotherapy had a late metastatic relapse
Summary
Extraskeletal Ewings sarcoma are rare tumors for which there is no consensus on optimal management. Ewing's family of tumors arise primarily from bones and rarely are of extraskeletal origin. Considering the rarity and varied presentation of extraskeletal Ewings sarcoma (ESES), no definite recommendation regarding optimal treatment has been defined. The principles of management of extraskeletal Ewings tumors have been extrapolated from experience from treating Ewings sarcoma of bony origin or primitive peripheral neurectodermal tumors (PPNET). ESES patients are offered combined modality treatment as for Ewing's sarcoma (ES) of the same site and stage, though some studies have suggested that extraosseous primaries may have a poorer prognosis[1]. We are presenting a single institution expe-. World Journal of Surgical Oncology 2007, 5:3 http://www.wjso.com/content/5/1/3 rience of management of this uncommon tumour along with a review of relevant literature
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
