主动脉瓣乳头状弹力纤维瘤1例报道并文献复习

Cardiac papillary fibroelastoma in aortic valve: a case report and review of literature

目的 探讨发生于心脏瓣膜的乳头状弹力纤维瘤的临床和病理学特征 ,为临床治疗提供依据。方法 应用光镜、组织化学、免疫组化方法对 1例发生于主动脉瓣的乳头状弹力纤维瘤进行形态学研究并复习文献。结果 心脏的乳头状弹力纤维瘤临床上缺乏特征性的症状和体征。心动超声提示主动脉瓣异常回声。组织学 :瘤组织呈乳头状生长 ,乳头中央为红染均质物 ,表面衬覆单层立方或扁平上皮。免疫组化 :乳头状表面衬覆的上皮CD31和CD34(+) ,乳头轴心则CD117和S 10 0蛋白 (+) ,弹力纤维染色 (+)。结论 发生于心脏的乳头状弹力纤维瘤十分罕见 ,主要发生于瓣膜表面。尽管在病理学方面根据此肿瘤的发生部位及组织学形态不难做出诊断 ,但因其临床症状不典型 。

Objective To investigate the clinical and pathological characteristics of the cardiac papillary fibroelastoma in artic valve and to discuss the possibility of the therapy. Methods Histological and specific immunhistochemical studies were conducted on the samples and review of the literatures were performed. Results The patient presented no significant clinical symptoms and syndrome of the cardiac papillary fibroelastoma. Cardiogram showed unusually echo on aortic valve. Histopathologically, the tumor tissue grown in papillary pattern. The red mass was obsevered in the center of the papilla. The papillary were covered with cubic and flat epithelium. The tumor covered with cubic and flat epithelium cells were positive for CD31 and CD34, while CD117 and S-100 protein were positive and elastic fiber was positive in the stroma of the tumor. Conclusion The cardiac papillary fibroelastoma is rare and formed mainly on the surface of the valves. It lacks typical clinical symptoms. Therefore, cardiac papillary fibroelastoma is not easy to diagnose clinically, although its location and histology is typical and easy to be recognized.