上皮样血管肉瘤4例临床病理分析

Epithelioid angiosarcoma:a clinicopathologic study of 4 cases

目的 探讨上皮样血管肉瘤的临床病理学特征。方法 对 4例上皮样血管肉瘤进行光镜和免疫组化检测 ,1例做电镜观察。结果 男性 3例 ,女性 1例 ,平均 6 1岁。头颈部 2例 ,膀胱 1例 ,上臂 1例。瘤细胞上皮样增生呈片状 ,细胞大 ,多角形 ,胞质丰富 ,嗜酸 ,核大 ,核仁明显 ,核分裂象易见 ,可见胞质内空泡形成 ,偶见含有单个红血球 ,多数病例能找到互相吻合的血管腔隙 ,其内衬非典型的内皮细胞 ,间质易见灶性淋巴细胞浸润和含铁血黄素。Vim、CD31、CD344例均 (+) ;FⅧAg及低分子角蛋白 (CK7) 2例 (+) ;S 10 0、HMB4 5、EMA、HHF35和SMA 4例均(- )。电镜观察 1例见胞质内含丰富的微丝和少许W P小体。 3例随访 1～ 3年 ,2例有转移 ,1例转移至局部淋巴结 ,1例转移至肺 ,1例术后半年死亡。结论 上皮样血管肉瘤是血管肉瘤的一种特殊类型 ,熟悉其形态特征 ,对避免误诊具有重要意义。

Objective To study the clinicopathological features of epithelioid angiosarcoma (EA) Methods Four cases of EA were examined by light microscopy and immunohistochemistry,and additional ultrastructural observation was performed in 1 case Results The patients' age ranged from 53 to 68 years (mean 61 years) Tumors presented at a variety of sites including head and neck (2 cases), bladder (1 case) and the upper arm (1 case). The morphological characteristics showed that these tumors were composed of a sheet like proliferaton of large polygonal epithelioid cells that had an abundant eosinophilic cytoplasm The nuclei were large and contained a prominent central nucleolus Mitotic figures were frequently seen Cytoplasmic vacuoles, some of which contained single red blood cell, could be observed Most of the cases had areas of conventional angiosarcoma with intercommunicating vascular channels lined by atypical endothelial cells Focal lymphocytes infiltration and deposition of hemosiderin might be easily seen in the stroma Immunohistochemical staining showed that tumor cells were positive for vimentin (4/4), CD31 (4/4) , CD34 (4/4), cytokeratin (2/4) and FⅧAg(2/4), but negative for S 100, HMB45,EMA,HHF35 and SMA Ultrastructural study of 1 case demonstrated Weibel Palade bodies and an abundance of intermediate filaments Three cases with follow up found that 2 cases developed metastasis, and 1 case died of tumor Conclusions The epithelioid angiosarcoma is a variant of angiosarcoma Recognizing its morphological characteristics is important to avoid confusion with other similar lesions.