Proximal-type epithelioid sarcoma: Case report of an unusual presentation

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• 作者：R.S.R. Woods^a ; ^{woodsr@tcd.ie} ; M.P. Dempsey^a ; H.F. Rizkalla^b ; M.E. McMenamin^b ; D. O&rsquo ; Donovan^a
• 关键词：Proximal-type epithelioid sarcoma ; Epithelioid sarcoma ; Limb sarcoma ; Metastatic sarcoma ; Plastic surgery
• 刊名：Journal of Plastic ; Reconstructive Aesthetic Surgery
• 出版年：2012
• 期刊代码：193_17486815
• 类别：med
• 出版时间：July, 2012
• 卷：65
• 期：7
• 页码：977-980
• 文件大小：969 K

摘要

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Summary

Epithelioid sarcoma, first described by Enzinger in 1970, classically presents in young adults and usually arises in the distal extremities. The proximal-type variant, first described in 1997 as a rare aggressive form of sarcoma, usually arises more proximally. It carries a higher mortality rate than classical limb epithelioid sarcoma and is often resistant to multimodal treatment. We report the case of a 27-year old male who had a delayed diagnosis of proximal-type epithelioid sarcoma of the forearm. This was originally thought to be a necrotising soft tissue infection and was unfortunately metastatic at the time of eventual diagnosis. The clinical and histopathological features of this challenging tumour are discussed and the relevant literature is reviewed.