心脏原发性血管肉瘤的临床及病理分析

Clinical and pathological analysis of primary cardiac angiosarcoma

目的 探讨心脏原发性血管肉瘤的临床及病理诊断和预后 ,为该肿瘤早期发现、早期诊断和早期治疗提供依据。方法 报导 1例发生在心脏的病例 ,从临床病理、鉴别诊断等方面进行分析并文献复习。结果 其临床主要表现为面部浮肿 ,全身乏力。UCG示右房内一约 5 .6cm× 4 .1cm的较致密低回声团块 ,活动度差。肿瘤暗红色 ,大部分表面光滑 ,少部分粗糙 ,切面暗红夹灰白色 ,质地较软。肿瘤主要为许多不规则的血管腔 ,部分区域呈自由交织的血管网 ,内衬异形的上皮细胞。免疫组化标记显示内衬上皮细胞CD34(+) ,VIII因子 (+) ,Vim(+) ,EMA(- )。结论 心脏原发性血管肉瘤是一种罕见的心脏肿瘤 ,预后很差。好发于中年男性 ,多侵犯右心房。UCG为临床早期发现、早期诊断的首选方法 ,并结合MRI全面了解该肿瘤向心腔内外扩展情况及肿瘤内部特征 ,有助于该肿瘤的鉴别诊断和外科手术方式的选择。

Objective To study the clinical and pathological diagnosis and the prognosis of primary cardiac angiosarcoma to provide data for its early discovery , diagnosis and treatment. Methods One case of primary cardiac angiosarcoma was reported with emphasis on its clinical pathologic features and differential diagnosis, as well as review of the literature. Results Facial edema and asthenia were the chief clinical symptoms. Rather compact and light echo lump of 5.6 cm×4.1 cm were found in UCG, which mobility was poor. The tumor was dark red, smooth and rough on the surface, dark red and pale in section. Microscopically, the tumor was mainly composed of distinct vascular channels of irregular sizes and shapes lined by atypia endothelium. Moreover, there was a tendency for the channels to connect with each other, creating an anastomosing network of sinusoids. The endothelium was positive for CD34, VIII factor, Vim and negative for EMA. Conclusion Primary cardiac angiosarcoma is a rare tumor, There is very poor prognosis. It has a predilection for middle-aged males and a marked predominance in the right atrium. UCG is the first method for early clinical findings and diagnosis. With MRI the extension of cardiac luminal inside and outside and the internal characteristics can be known comprehensively, which can also contribute to the differential diagnosis and the selection of surgical operative pattern.