原发性心脏瓣膜肿瘤临床病理观察

目的分析心脏瓣膜原发肿瘤病理学特点及其与临床表现的关系。方法收集经外检确诊的心脏瓣膜原发肿瘤11例,HE、Weigert—Van Gieson染色观察,同时部分病例加做免疫组织化学（SP法）。结果心脏瓣膜原发肿瘤较为罕见,仅占心脏原发肿瘤的3％（11／426）,其中良性肿瘤占多数（10／11）,恶性少见（1／11）。肿瘤类型以乳头状弹力纤维瘤（4／11）和海绵状血管瘤最为多见（4／11）,血管球瘤1例,血管肉瘤1例,纤维性错构瘤1例。肿瘤发生部位以房室瓣多见,三尖瓣4例,二尖瓣4例,肺动脉瓣2例,主动脉瓣1例。7例术前心脏超声心动图诊断为瓣膜肿瘤,4例误诊或漏诊。结论临床对于心脏瓣膜原发肿瘤的诊断比较困难,影像学缺乏对心脏瓣膜原发肿瘤的认识。虽然良性肿瘤的手术切除预后较好,但可导致血流动力学改变而致命,应尽早诊断及手术治疗。术中冷冻切片检查对临床决定手术方式及后期治疗有指导意义。

Clinicopathologic studies of 11 cases of primary cardiac valve tumors

Objective To study the clinicopathologic features of primary cardiac valve tumors. Methods Eleven cases of primary valve tumors collected from Fuwai Hospital during the period from 1983 to 2005 were enrolled into the study. The tumors were stained with hematoxylin and eosin and Weigert-Van Gieson stain. Immunohistochemistry was also carried out in selected examples. Results Primary cardiac valve tumors were uncommon and accounted for only 3% (11/426) of all primary cardiac tumors. Most of them (10/11) were benign and malignancy was rarely encountered (1/11). The tumor subtypes included papillary fibroelastoma (4/11), cavernous hemangioma (4/11), glomus tumor (1/11), angiosarcoma (1/11) and hamartoma (1/11). Of the 11 tumors studied, 4 involved the tricuspid valve, 4 involved the mitral valve, 2 involved the pulmonary valve and 1 involved the aortic valve. The diagnosis was established by preoperative echocardiography in 7 patients. The remaining 4 cases were either misdiagnosed or undiagnosed. Conclusions Preoperative diagnosis of primary cardiac valve tumors can be difficult due to lack of detailed information related to this group of lesions. Although benign cardiac valve tumors carry a good prognosis, the clinical outcome may be disastrous as a result of hemodynamic disturbances. Intraoperative frozen section examination is advisable for guiding proper surgical management.