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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 clinicopathological characteristics and diagnosis of primary non-myxomatous neoplasms and to improve physician’s understanding of these tumors and to improve their diagnostic value. Methods: A retrospective study of 19 cases of primary cardiac non-myxomatous neoplasm resected from January 2009 to July 2016 in Ruijin Hospital Affiliated to Shanghai Jiaotong University School of Medicine was conducted. The clinical history data, pathomorphological characteristics, Immunohistochemistry (immunohistochemistry) markers, and review the relevant literature at home and abroad to explore. Results: Of the 19 patients with primary non-myxomatous neoplasms, 9 were benign tumors, including 3 hemangiomas, 3 lipomas, 2 papillary fibroids and 1 schwannoma. Among them, 8 were malignant tumors, Including 5 cases of angiosarcoma, 1 case of malignant mesothelioma, 1 case of synovial sarcoma and 1 case of diffuse large B cell lymphoma; 2 cases of intermediate tumors, including 1 case of fibromatosis and 1 case of combined hemangioendothelioma. The clinical manifestations of most patients with repeated chest tightness and shortness of breath, the clinical symptoms of patients with benign tumors is relatively light, part of the occasional physical examination found that patients with severe clinical symptoms of malignant tumors. Conclusions: The incidence of primary non-myxomatous neoplasms is low, the tumor types are multifarious, and the clinical manifestations of patients are characterized by a lack of characteristics. The diagnosis of benign neoplasms is relatively easy. The malignant neoplasms are mostly soft tissue sarcomas, especially those with angiosarcomas. Other types Malignant tumors are rare, and the histological morphology is complex and diverse. The diagnosis depends on the histopathological and immunohistochemical examinations of the surgical specimens. If necessary, the molecular diagnosis should be performed.