Journal
PEDIATRIC BLOOD & CANCER
Volume 66, Issue 9, Pages -Publisher
WILEY
DOI: 10.1002/pbc.27879
Keywords
adolescents and young adults; children; CWS group; epithelioid sarcoma; surgery
Categories
Funding
- Federal Ministry of Research and Technology [01ZP0831]
- German Cancer Aid Foundation, Bonn, Germany [M34/87/Tr1, M76/91/Tr2, CWS-96:T9/96/TrI, CWS-2002P: 50-2721-Tr2]
- Deutsche Kinderkrebsstiftung, Bonn, Germany [A2007/13DKS2009.08]
- Foerderkreis Krebskranke Kinder Stuttgart, Germany
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Background Epithelioid sarcoma (ES) is a rare malignant soft-tissue tumor. Little is known about the optimal treatment of primary localized (LD), metastatic (MD), and relapsed disease (RD). Methods Characteristics, treatment, and outcome of 67 patients registered within the Cooperative Weichteilsarkom Studiengruppe CWS-81, -86, -91, -96, -2002P trials and the registry SoTiSaR were analyzed (1981-2016). Results The median age was 14 years (range, 0.7-26.9); 53 patients had localized disease (LD) and 14 metastatic disease (MD). A total of 58 of 67 patients were treated with primary resection. Resection was microscopically complete (R0) in 35, microscopically incomplete (R1) in 12, macroscopically incomplete (R2) in 20 patients. Radiotherapy (RT) was administered to 33 of 67 patients and 49 of 67 patients received chemotherapy (CHT). Complete remission (CR) was achieved in 45 of 53 (85%) patients with LD. Twenty-seven of 53 patients relapsed after a median time of 0.9 years (range, 0.1-2.3). Relapse therapy consisted of resection (n = 19/27), RT (n = 10/27), CHT (n = 12/27), and limb perfusion (n = 3/27). The five-year event-free survival and overall survival of patients with LD, MD, and RD was 35% (+/- 12, CI 95%) and 58% (+/- 14, CI 95%), 7% (+/- 14, CI 95%), and 9% (+/- 16, CI 95%), 24% (+/- 17, CI 95%), and 40% (+/- 20, CI 95%), respectively. Tumor size, IRS group, tumor invasiveness, nodal status, and best resection correlated with a favorable prognosis in patients with LD while best resection was the only significant factor in patients with RD. Conclusions Complete tumor resection correlates with long-term survival in patients with ES.
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