心脏原发性非黏液瘤性肿瘤19例临床病理分析  

Primary non-myxomatous cardiac tumor: clinicopathologic analysis of 19 cases

作　　者：陈晓炎[1] 王朝夫[1] 董磊[1] 许海敏[1] 金晓龙[1] 

机构地区：[1]上海交通大学医学院附属瑞金医院病理科,上海200025

出　　处：《诊断学理论与实践》2017年第1期66-72,共7页Journal of Diagnostics Concepts & Practice

摘　　要：目的:探讨心脏原发性非黏液瘤性肿瘤的临床病理特征及诊断要点,加深医师对这类肿瘤的认识,提高诊断水平。方法:回顾性研究2009年1月至2016年7月于上海交通大学医学院附属瑞金医院手术切除的19例心脏原发性非黏液瘤性肿瘤病例,分析其临床病史资料、病理形态学特点及免疫组织化学(免疫组化)标记特征,并复习国内外相关文献进行探讨。结果:19例心脏原发性非黏液瘤性肿瘤中,良性肿瘤9例,包括3例血管瘤,3例脂肪瘤,2例乳头状弹力纤维瘤和1例神经鞘瘤;恶性肿瘤8例,包括5例血管肉瘤,1例恶性间皮瘤,1例滑膜肉瘤和1例弥漫性大B细胞淋巴瘤;中间型肿瘤2例,包括1例纤维瘤病和1例组合性血管内皮瘤。多数患者临床表现为反复胸闷气促,良性肿瘤患者临床症状相对轻,部分为体检中偶然发现,恶性肿瘤患者临床症状较重。结论:心脏原发性非黏液瘤性肿瘤发病率低,肿瘤类型多,患者临床表现缺乏特征性,其中良性肿瘤的诊断相对容易;而恶性肿瘤主要为软组织肉瘤,尤其以血管肉瘤居多,其他类型恶性肿瘤罕见,且组织学形态复杂多样,明确诊断依靠手术标本的病理组织学检查及免疫组化检查,必要时需行分子检测辅助诊断。Objective： To investigate the clinicopathologic and diagnostic features of non-myxomatous cardiac tumors for improving the diagnostic level of the disease. Methods： Nineteen patients with pathologically confirmed primary non- myxomatous cardiac tumor during January 2009 to July 2016 at Ruijin Hospital were enrolled in this study. The clinical and pathologic features, imaging findings and immunophenotype of these cases were analyzed retrospectively and relevant literatures were reviewed. Results： Nine of the 19 cases were benign tumor （9/19）, including 3 hemangioma, 3 lipoma, 2 papillary fibroelastoma and 1 schwannoma. Eight cases were malignant tumors （8/19）, including 5 angiosarcoma, 1 malig- nant mesothelioma, 1 synovial sarcoma and 1 diffuse large B cell lymphoma.Two cases were intermediate tumors （2/19）, including 1 fibromatosis and 1 composite hemangioendothelioma. Clinically, most of the patients presented with recurrent chest tightness and dyspnea. In patients with benign tumor, symptoms was mild or even absent and some patients were dis- covered during routine physical examination. Serious symptoms were seen in patients with malignant tumor. Conclusions： Primary cardiac non-myxomatou~,,tumors are extremely rare,consisting various types of tumors and lacking specific clini- cal manifestations. The diagnosis of benign tumor is relatively easier. The malignant tumors are mainly sarcoma, especially angiosarcoma. Other types of malignant tumors are rare and their histomorphology are of diversive state; the definite di- agnosis depends on histopathology and immunohistochemical examination and molecular detection.

关 键 词：心脏原发性肿瘤 非黏液瘤性肿瘤 组织病理 诊断 

分 类 号：R732.1[医药卫生—肿瘤]