Sassi S H, Dhouib R, Ben Dhaou S, Mrad K, Driss M, Abbes I, Arbi H, Haourai H, Ben Romdhane K
Service d'anatomie et de cytologie pathologiques, institut Salah-Azaeiz, Bab-Saadoun, 1006 Tunis, Tunisie.
Rev Chir Orthop Reparatrice Appar Mot. 2008 Feb;94(1):92-5. doi: 10.1016/j.rco.2007.10.004. Epub 2008 Feb 20.
Sclerosing epithelioid fibrosarcoma is a rare variant of fibrosarcomas, which was recently identified as a separate entity due to specific histologic and immunohistochemistry features and its poor prognosis. We report a case of sclerosing epithelioid fibrosarcoma of soft tissues, which developed in a 37-year-old woman who presented a tumor involving the posteromedial aspect of the left knee and which progressed in size for one year. Imaging revealed a well-delimited tumor process measuring 8 cm in its largest diameter and situated in the medial compartment of the left knee. Histology of the tumorectomy specimen and the immunohistochemistry study led to the diagnosis of sclerosing epithelioid fibrosarcoma of soft tissues. This new case illustrates the characteristic features of this tumor and recalls the difficult pathological diagnosis.
硬化性上皮样纤维肉瘤是纤维肉瘤的一种罕见变体，由于其特定的组织学和免疫组化特征以及预后不良，最近被确认为一个独立的实体。我们报告一例软组织硬化性上皮样纤维肉瘤病例，该病例发生在一名37岁女性身上，她的左膝后内侧出现肿瘤，且肿瘤大小在一年内不断增大。影像学检查显示一个边界清晰的肿瘤，最大直径为8厘米，位于左膝内侧间室。肿瘤切除标本的组织学检查和免疫组化研究确诊为软组织硬化性上皮样纤维肉瘤。这个新病例说明了这种肿瘤的特征，并再次强调了其病理诊断的困难。
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