Surgery Department, Gynecology Section and Obstetrics, Tor Vergata University, Rome, Italy.
Diagn Pathol. 2013 Jul 25;8:122. doi: 10.1186/1746-1596-8-122.
The "proximal-type" epithelioid sarcoma is a very rare kind of mesenchimal tumor characterized by the difficulty in histological diagnosis and the very aggressive biological behavior.
We report of a case of a 63 years old woman with a vulvar "proximal-type" epithelioid sarcoma that underwent a radical surgical staging followed by an adjuvant radiotherapy. She is on follow-up care for 14 months and there is no clinical evidence of disease.
Even if quite rare the proximal type epithelioid sarcoma should be regarded as a separate entity of particularly aggressive biologic behaviour. Its diagnosis attracts controversies and criticism related to the surgical approach and the choice of an adjuvant therapy.
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“近端型”上皮样肉瘤是一种非常罕见的间叶组织肿瘤，其组织学诊断困难，生物学行为极具侵袭性。
我们报告了一例 63 岁女性外阴“近端型”上皮样肉瘤患者，接受了根治性手术分期，随后进行了辅助放疗。患者目前已随访 14 个月，无临床疾病证据。
即使非常罕见，近端型上皮样肉瘤也应被视为一种具有特别侵袭性生物学行为的独立实体。其诊断存在争议，涉及手术方法和辅助治疗的选择。
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