静脉内平滑肌瘤病心脏受累的声像图特征

Ultrasonic Characteristics of Intravenous Leiomyomatosis with Intracardiac Extension

目的探讨静脉内平滑肌瘤病(IVL)心脏受累的超声特点,提高对该病的认识和诊断水平。方法回顾性分析13例经手术病理证实的IVL心脏受累患者的临床资料和声像图特点。13例患者均为女性,10例为首次发病,3例为复发患者;8例既往因子宫平滑肌瘤行子宫全切或部分切除术。结果 5例患者首发症状表现为活动后胸闷气短,4例出现腹胀、下肢水肿,3例出现活动后心悸,1例出现月经过多,另有3例无明显自觉症状。超声表现为:13例患者均可见右心腔(62%位于右心房,38%位于右心房和右心室)和下腔静脉相连的中低回声占位性病变,其中右心腔占位9例为团块状、4例为条索状;10例患者伴有盆腔或子宫低回声或混合性回声占位,其中60%病灶可见丰富血流。结论 IVL心脏受累具有一定的声像图特征,超声检查对该病诊断具有重要价值。

Objective To explore the sonographic characteristics of intraveous leiomyomatosis （IVL） with intracardiac extension and improve its diagnosis. Methods The clinical and sonographic data of 13 female patients with pathologically confirmed IVL with intracardiac extension who were treated in our hospital between 2002 and 2012 were retrospectively analyzed. These patients aged 44 years old （range： 38-49 years） , and 10 of them were first-episode patients and the remaining 3 were recurrent patients. Eight patients had a his- tory of hysterectomy for leiomyoma. Results The first-episode symptoms included exertional chest tightness and shortness of breath （ n = 5 ） , abdominal distention and edema of low extermity （ n = 4 ）, exertional palpitation of cardiac origin （ n = 3 ）, and menorrhagia （ n = 1 ）. Ultrasonography showed that all patients had isoechoic or hypoechoic tumors extended through the inferior vena cava into right heart chambers （ 62% in right atrium alone and 38% in right ventricle and atrium）. Nine masses in right heart chamber （69.2%） were oval and 4 （30.8%） were serpentine, which were all with well-demarcated borders and most （80%） with heteroechogenic texture. Ten patients had hypoechoic or mixed echoic tumors in pelvic cavity or uterus, and 6 of them had abundant blood flow. Conclusions IVL with intracardiac extension has certain sonographic characteristics Ultrasonography is a valuable tool in the diagnosis of IVL with intracardiac extension.