论文部分内容阅读
目的探讨骨原发性上皮样血管内皮瘤(EHE)的临床病理特征及诊断和鉴别诊断,以提高对该病变的认识。方法观察4例骨原发性EHE的临床病理特征及免疫组化表型特征,并结合文献分析讨论。结果骨EHE好发于年轻男性,男女之比为2∶1。主要临床表现为局部疼痛,其临床病程长短不一,可复发转移。病变以多中心性居多,X线下为无特征性的溶骨性病变。镜下上皮样瘤细胞呈条索状或巢团状分布于黏液样或透明变性的间质中,形成不同发育阶段的血管,可见单细胞血管腔。免疫组化:瘤细胞CD34、CD31、F8因子、vimentin和广谱CK(+)。结论骨EHE是一种少见的血管源性恶性肿瘤,具有独特的临床病理学特征及预后,临床及影像学诊断有困难,病理学检查是最佳诊断手段,应注意与感染、转移癌及其他良性病变相鉴别。
Objective To investigate the clinicopathological characteristics, diagnosis and differential diagnosis of primary epithelial hemangioendothelioma (EHE) in bone so as to improve the understanding of this lesion. Methods The clinicopathological and immunohistochemical features of 4 cases of primary EHE were observed and analyzed. Results Bone EHE occurred in young men, male to female ratio of 2: 1. The main clinical manifestations of local pain, the clinical course of varying lengths, recurrence and metastasis. The majority of lesions to multi-center, X-ray characteristic osteolytic lesions. Microscopic epithelioid tumor cells were cords or nest clusters distributed in the mucus-like or transparent degeneration of the stroma, the formation of blood vessels at different stages of development, showing single-cell vascular lumen. Immunohistochemistry: tumor cells CD34, CD31, F8 factor, vimentin and broad-spectrum CK (+). Conclusion Bone EHE is a rare malignant tumor of vasculature. It has unique clinicopathological features and prognosis. It has difficult clinical and imaging diagnosis. Pathological examination is the best diagnostic method. It should pay attention to infection, metastasis and other Differentiation of benign lesions.