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目的探讨骨原发性恶性上皮样血管肿瘤的临床病理特征、诊断及鉴别诊断。方法对4例骨原发性上皮样血管内皮细胞瘤(EHE)和2例上皮样血管肉瘤(EA)进行临床、光镜及免疫组化分析。结果 4例EHE中3例行刮除术,1例行化学治疗,均存活。2例EA分别行单纯化疗和手术治疗,1例失访,1例2个月后死亡。镜检:EHE的肿瘤细胞呈巢状、片状或条索状排列,间质常黏液样变性或透明变性,瘤细胞轻度异型,常见胞质内空泡。EA主要由呈实性片状、巢状排列的上皮样肿瘤细胞组成,瘤细胞异型性大,核呈空泡状,核仁显著,核分裂易见。免疫组化:瘤细胞CD31和vimentin(+)。结论 EHE低度恶性,EA高度恶性,两者鉴别主要依靠病理学特征,治疗和预后均差异较大,准确诊断是正确治疗的前提。
Objective To investigate the clinicopathological features, diagnosis and differential diagnosis of primary malignant epithelioid bone tumor. Methods Four cases of primary epithelioid hemangioendothelioma (EHE) and two cases of epithelioid angiosarcoma (EA) were studied by clinical, light microscopy and immunohistochemistry. Results In 4 cases of EHE, 3 patients underwent curettage and 1 patient underwent chemotherapy, all surviving. Two cases of EA were treated with chemotherapy and surgery alone, one case was lost, one case died after 2 months. Microscopic examination: EHE tumor cells were nested, lamellar or cord-like arrangement, often mucinous or interstitial degeneration or degeneration, mild dysmorphic tumor cells, common cytoplasmic vacuoles. EA mainly consists of solid sheet-like, nested arrangement of epithelioid tumor cells, tumor cells atypia, vacuolar nucleus, prominent nucleoli, nuclear fission is easy to see. Immunohistochemistry: tumor cells CD31 and vimentin (+). Conclusions EHE is highly malignant and has a high degree of malignancy. The differential diagnosis of EHE mainly depends on the pathological features, and the treatment and prognosis are quite different. Accurate diagnosis is the prerequisite for correct treatment.