A single institution experience of combined modality management of extra skeletal Ewings sarcoma
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A single institution experience of combined modality management of extra skeletal Ewings sarcoma Ramachandran Venkitaraman1, Mathew K George*2, S Ganapathy Ramanan3 and TG Sagar2 Address: 1Division of Clinical Oncology, Royal Marsden Hospital, Sutton, Surrey, SM2 5PT, UK, 2Division of Medical Oncology, Royal Darwin Hopsital, Casuarina, Australia and 3Division of Medical Oncology, Cancer Institute(WIA), Adyar, Chennai, 400036, India Email: Ramachandran Venkitaraman - [email protected]; Mathew K George* - [email protected]; S Ganapathy Ramanan - [email protected]; TG Sagar - [email protected] * Corresponding author
Published: 11 January 2007 World Journal of Surgical Oncology 2007, 5:3
doi:10.1186/1477-7819-5-3
Received: 25 September 2006 Accepted: 11 January 2007
This article is available from: http://www.wjso.com/content/5/1/3 © 2007 Venkitaraman et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract Background: Extraskeletal Ewings sarcoma are rare tumors for which there is no consensus on optimal management. Methods: A 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. Results: A 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). Conclusion: Aggressive 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.
Background 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 extrapo-
lated from experience from treating Ewings sarcoma of bony origin or primitive peripheral neurectodermal tumors (PPNET). Commonly ESES patients are offered combined modality treat
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