伴有心脏受累静脉内平滑肌瘤病.docVIP

伴有心脏受累静脉内平滑肌瘤病[摘要] 目的:探讨伴有心脏受累的静脉内平滑肌瘤病的临床及病理形态特点。方法:观察1例伴有心脏受累的静脉内平滑肌瘤病的病理形态(光镜、免疫组化)及临床资料分析,并结合文献讨论。结果:光镜下心房内肿瘤与右附件区肿瘤光镜下病理学形态完全一致,免疫组织化学证实为平滑肌来源。结论:静脉内平滑肌瘤病是一种特殊类型的子宫肌瘤,多发生于子宫肌壁间的静脉内,偶可延伸至下腔静脉甚至右心房,具有潜在的致命性。 [关键词] 平滑肌瘤病;心房 [中图分类号] R685.6 [文献标识码]A [文章编号]1674-4721(2010)04(a)-018-02 Intravenous leiomyomatosis with intracardiac extension WANG Yufeng1,LI Liang2 (1.Jining Hospital for Prevention and Save of the Skin Diseases,Jining 272000, China;2.No.1 Affiliated Hospital of Jining Medical College,Jining 272000, China) [Abstract] Objective: To investigate cardiac involvement associated with intravenous leiomyomatosis of the clinical and pathological features. Methods: One cases of cardiac involvement associated with intravenous leiomyomatosis of the pathological changes(light microscopy,immunohistochemistry) and clinical data analysis,combined with literature discussion. Results: By light microscope, the right atrial tumor and the tumor in the annex had the same pathology light microscope,immunohistochemistry confirmed smooth muscle origin. Conclusion: Intravenous leiomyomatosis is a special type of uterine fibroids,mostly occurs in the intramural uterine veins,the dual can be extended to the inferior vena cava or right atrium, with potentially lethal. [Key words] Leiomyomatosis;Atrial 静脉内平滑肌瘤病是一种特殊类型的子宫肌瘤,其生长方式类似恶性肿瘤,达下腔静脉甚至累及右心腔者非常罕见,具有潜在的致命性。本文报道1例伴有心脏受累的静脉内平滑肌瘤病患者,并结合文献,就其主要病理改变和临床特点进行讨论。 1 资料与方法 患者,女性,52岁。患者3个月前因“面部潮红,水肿”来本院就诊,B超发现盆腔肿物,遂入院要求手术治疗,入院行妇科检查,子宫及双侧附件B超:子宫前位,大小6.3 cm×5.2 cm,肌层回声尚均匀,宫腔线欠清晰,内膜厚约0.5 cm,右附件区见8.6 cm×7.4 cm的不均匀团块,边界尚清,形态不规则,内见丰富血流信号,最大流速32.01 cm/s,RI:0.54,左侧附件区未见明显异常。患者术前心电图:①不完全性右束支传导阻滞;②ST-T改变;③左胸导联低电压。心脏彩超示:右房室内径扩大,余各房室腔大小形态正常,室间隔及左室后壁厚度正常,静息状态下未见明显节段性室壁运动异常。右房内见7.5 cm×4.5 cm实性团块,随心脏收缩与扩张往返于右房室之间。各瓣膜形态结构及启闭功能未见明显异常,偶见心律不齐。升主动脉及主动脉内径正常;心包腔内未探及明显积液征象。CDFI:三尖瓣开放时与瓣口可见五彩镶嵌血流信号。超声提示:右房室扩大;右房黏液瘤可考虑;假性三尖瓣狭窄;心律不整。考虑到患者心脏与子宫均有肿瘤,经院内会诊决定先行心脏肿瘤切除,故患者转心胸外手术治疗,术中见右心房内有1枚10.0 cm×7.0 cm×6.0 cm大小肿物,表面光滑(图1)。临床诊断:心脏黏液瘤。患者术后3个月在