Abstract
目的： 探讨原发性心脏血管肉瘤（primary cardiac angiosarcoma，PCAS）临床病理及遗传学特点。 方法： 收集河南省人民医院确诊的PCAS共9例，采用免疫组织化学和二代测序技术检测蛋白和基因突变情况。 结果： 本组患者男性7例，女性2例；年龄18~53岁；6例位于右心房，2例位于心包，1例位于右房室沟。9例伴心包积液，3例伴胸腔积液，4例伴肺多发转移。细胞学见肿瘤细胞呈腺样、乳头状排列，上皮样形态，酷似腺癌细胞；组织学见肿瘤组织呈高-中等分化，见不规则血管腔样结构，瘤细胞鞋钉样或乳头状，部分区域呈片状、束状排列，细胞胖梭形，核深染不规则，异型性明显，病理性核分裂象易见；免疫标记显示肿瘤细胞强表达CD34、CD31、ERG。伴多发肺转移结节和浆膜腔积液病例存在TP53错义突变（p.R273C），且肿瘤细胞p53蛋白强阳性。 结论： PCAS早期即存在浆膜腔积液和肺多发转移，组织形态呈高-中等分化，细胞学极易误诊为腺癌，免疫组织化学有助于鉴别诊断；且TP53突变可能与肿瘤的高侵袭性生物学行为相关。.
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Schedule a callMore From: Zhonghua bing li xue za zhi = Chinese journal of pathology
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