摘要目的 分析心脏黏液纤维肉瘤的临床病理特征.方法 收集北京安贞医院2009年1月至2014年12月间所有5例心脏黏液纤维肉瘤病例,对其临床资料、病理形态学、免疫组织化学特征进行观察,并进行文献复习.结果 5例心脏黏液纤维肉瘤患者中女性4例,男性1例;年龄39~61岁;平均年龄(50.4±9.0)岁.肿瘤均主要位于左心房,广基隆起的肿块,其中1例浸透心脏,局部浸润左下肺组织.组织形态学谱分级,1例为低级别,1例为中级别,3例为高级别.根据法国国家联邦癌症中心分级系统,1例1级,4例2级.免疫组织化学染色5例波形蛋白均弥漫强阳性,不同程度的平滑肌肌动蛋白、肌特异性肌动蛋白阳性.其中4例手术切除大部分肿物,1例完全切除肿物.术后随访到3例,1例完全切除肿物病例术后生存13个月,2例部分切除肿物病例均术后1个月死亡.结论 心脏黏液纤维肉瘤最多见于左心房.组织学上部分形态学良善的黏液纤维肉瘤容易被误诊为黏液瘤,诊断时需注意鉴别.精确地影像学分析,多学科合作,充足的初始外科治疗,可有助于提高黏液纤维肉瘤患者预后.

abstractsObjective To investigate the clinicopathologic features of cardiac myxofibrosarcomas. Methods The clinical data, pathomorphologic and immunohistochemical features were evaluated in five cases of cardiac myxofibrosarcoma collected from January 2009 to December 2014, with relevant literature review. Results Five patients with cardiac myxofibrosarcoma, including four women and one man [ age range 39-61 years; mean ( 50. 4 ± 9. 0 ) years ] were included. All tumors were broadbased and located mainly in the left atrium, with one case extending through the atrial wall and pericardium to the left lower lung lobe. The morphological grade was low in one case, intermediate in one, and high in three. Using Fédération Nationale des Centres de Lutte Contre le Cancer ( FNLCC) grading system, one case was grade 1 and four cases were grade 2. Immunohistochemical analysis revealed diffuse and strong expression for vimentin in all cases. Smooth muscle actin and muscle specific actin were variably expressed. Complete tumor excision was performed in one case, and tumor debulking was performed in the other four cases. Clinical follow-up was available in three cases. One patient with en bloc excision of the tumor mass survived 13 months and the other two with tumor debulking died one month after surgery. Conclusions The most common location for cardiac myxofibrosarcoma is the left atrium. Some myxofibrosarcoma may be histologically bland and misdiagnosed as myxoma due to histological similarities. Thus caution should be exercised in their microscopic differentiation. Precise imaging, multidisciplinary approach and adequate initial surgery may contribute to improving the clinical outcomes of myxofibrosarcoma.