侵犯心脏的静脉内平滑肌瘤病诊疗经验

目的 探讨侵犯心脏的静脉内平滑肌瘤病的诊断特点和治疗方法.方法 回顾性分析北京大学人民医院血管外科2002年11月至2011年2月收治的8例心脏受累的静脉内平滑肌瘤病患者临床资料、手术方案和随访情况.结果 8例患者影像学或手术证实病变起源于盆腔,下腔静脉、心脏受累,无肺栓塞.3例表现为心悸、胸闷等心脏症状,4例表现为下肢水肿、腹胀等下腔静脉回流障碍症状,1例无明显临床症状.8例患者均有子宫肌瘤病史,6例既往曾行子宫切除术.5例在体外循环下行右心、下腔静脉内肿瘤切除,盆腔受累静脉结扎术;2例行非体外循环下腔静脉、盆腔肿瘤切除术;姑息性激素治疗1例.手术患者中2例先期在体外循环下行右心、下腔静脉内肿瘤切除术,3个月后行子宫、双附件及病变阔韧带切除术,未行激素治疗,其余5例患者接受大剂量激素治疗.超声随访15～90个月发现未行激素治疗组和激素治疗组均有2例复发.结论 侵犯心脏的静脉内平滑肌瘤病较为罕见,手术完整切除病变是治疗关键,激素治疗预防复发效果有限.

Abstract：

Objective To explore the diagnostic features and therapeutic methods of intravenous leiomyomatosis with a potential of heart invasion.Methods Eight cases of cardiac involvement with intravenous leiomyomatosis treated at our hospital from November 2002 to August 2011 were analyzed and compared. Results Either imaging or surgery confirmed that in all 8 patients the pelvic lesions originated from the inferior vena cava and heart involvement without pulmonary embolism. In 3 cases, palpitations and chest tightness were noticed. Four cases showed lower extremity edema and abdominal distension while no obvious clinical symptom was found for 1 case. All 8 cases had a history of uterine fibroids and 6 underwent previous hysterectomy. Among 8 patients, there were 5 cases of cardiopulmonary bypassing right heart, inferior vena cava tumor resection and pelvic involvement vein ligation and 2 cases of routine off-pumping of inferior vena cava with pelvic tumor resection. Palliative therapy was administered in 1 case. Two patients were resection the right heart and inferior vena cava tumor in the cardiopulmonary bypass, 3 months later were resection gynecologic uterine, double-accessories and broad ligament and were not treated with hormone.After operation, another 5 surgery patients received high-dose hormone treatments. 2 cases of recurrence in each group were found by ultrasound follow-ups from 15-90 months. Conclusion Invasion of the heart intravenous leiomyomatosis is a rare case, which can be tackled with a good effect by completely surgical resection and a limited effect on hormone therapy to prevent recurrence.

Surgical strategies on intravenous leiomyomatosis invading heart

Objective To explore the diagnostic features and therapeutic methods of intravenous leiomyomatosis with a potential of heart invasion.Methods Eight cases of cardiac involvement with intravenous leiomyomatosis treated at our hospital from November 2002 to August 2011 were analyzed and compared. Results Either imaging or surgery confirmed that in all 8 patients the pelvic lesions originated from the inferior vena cava and heart involvement without pulmonary embolism. In 3 cases, palpitations and chest tightness were noticed. Four cases showed lower extremity edema and abdominal distension while no obvious clinical symptom was found for 1 case. All 8 cases had a history of uterine fibroids and 6 underwent previous hysterectomy. Among 8 patients, there were 5 cases of cardiopulmonary bypassing right heart, inferior vena cava tumor resection and pelvic involvement vein ligation and 2 cases of routine off-pumping of inferior vena cava with pelvic tumor resection. Palliative therapy was administered in 1 case. Two patients were resection the right heart and inferior vena cava tumor in the cardiopulmonary bypass, 3 months later were resection gynecologic uterine, double-accessories and broad ligament and were not treated with hormone.After operation, another 5 surgery patients received high-dose hormone treatments. 2 cases of recurrence in each group were found by ultrasound follow-ups from 15-90 months. Conclusion Invasion of the heart intravenous leiomyomatosis is a rare case, which can be tackled with a good effect by completely surgical resection and a limited effect on hormone therapy to prevent recurrence.