原发性胸膜上皮样血管肉瘤2例临床病理分析
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摘要
目的探讨原发性胸膜上皮样血管肉瘤(epithelioid angiosarcoma, EAS)的临床病理学特征、鉴别诊断、治疗及预后。方法收集2例原发性胸膜EAS的临床病理资料,并复习相关文献。结果 2例患者男、女性各1例,年龄分别为47、45岁,均以呼吸系统症状就诊。影像学发现左侧胸膜增厚或包块。镜检见异型上皮样细胞及含红细胞的空泡状瘤细胞,胞质嗜伊红色或透明,胞核圆形空泡状或不规则形,核仁增大,局部见梭形瘤细胞,核分裂象5~12/HPF,坏死多少不等。瘤组织排列呈实性巢片状、条索状、窦隙状脉管样及乳头状。免疫表型:肿瘤细胞vimentin、CD31、ERG弥漫阳性,CD34阴性,Ki-67增殖指数为40%~60%。患者未行放、化疗,均于确诊后短期内死亡。结论原发性胸膜EAS十分罕见,其高度恶性、预后差,临床表现缺乏特征性,易误诊,组织病理学检查可确诊,需与转移癌、恶性间皮瘤、上皮样血管内皮瘤、黑色素瘤及大细胞淋巴瘤鉴别。
        
引文
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