Lian D W Q, Chuah K L, Cheng M H W, Yap W M
Department of Pathology, Tan Tock Seng Hospital, Singapore.
J Clin Pathol. 2008 Oct;61(10):1127-9. doi: 10.1136/jcp.2008.060137.
Perivascular epithelioid cell tumour (PEComa) is a term applied to a family of mesenchymal tumours composed of varying proportions of spindle and epithelioid cell components associated with HMB-45 expression. PEComa rarely arises in the soft tissue, visceral organs, skin and bone. This report details an instance when a purely epithelioid PEComa arose from the right fibula of a 52-year-old Chinese woman without features of tuberous sclerosis complex. The excision specimen disclosed an epithelioid tumour with a nested pattern associated with areas of nuclear pleomorphism, mitotic activity, necrosis and vascular invasion in addition to HMB-45 expression on immunohistochemistry. To the best of the authors' knowledge, this represents the first case of a histologically malignant PEComa of the bone. A short review of primary bone PEComas and potential problems in diagnosis is presented.
血管周上皮样细胞瘤（PEComa）是一个用于描述一类间充质肿瘤的术语，这类肿瘤由不同比例的梭形细胞和上皮样细胞成分组成，并伴有HMB-45表达。PEComa很少发生于软组织、内脏器官、皮肤和骨骼。本报告详细描述了一例发生于一名52岁中国女性右腓骨的纯上皮样PEComa病例，该患者无结节性硬化症复合体的特征。切除标本显示为上皮样肿瘤，呈巢状结构，除免疫组化显示HMB-45表达外，还伴有核异型性、有丝分裂活性、坏死和血管侵犯区域。据作者所知，这是首例组织学上为恶性的骨PEComa病例。本文还对原发性骨PEComa及诊断中的潜在问题进行了简要综述。
