伴有心脏受累的血管内平滑肌瘤病一例临床分析  

Intravenous leiomyomatosis with intracardiac involvement: a case report

作　　者：夏梦[1] 陈艳[2] 刘军秀[1] 王晶[1] 何勉[1] 

机构地区：[1]中山大学附属第一医院妇产科,广州510080 [2]中山大学附属第三医院药剂科,广州510630

出　　处：《新医学》2013年第12期861-865,共5页Journal of New Medicine

基　　金：国家临床重点专科2010

摘　　要：伴有心脏受累的血管内平滑肌瘤病(ICL)较罕见,术前诊断率低。该文报道了1例52岁ICL患者。其全身PET-CT检查提示子宫体部见多发低密度灶,形态欠规则,边界欠清,以子宫体左侧壁范围最大,约4.0 cm×2.0 cm×3.2 cm,病变延子宫阔韧带累及左侧附件;左卵巢静脉、左肾静脉管腔及下腔静脉增粗,其内见低密度影,考虑子宫平滑肌瘤病并通过左卵巢静脉、左肾静脉延伸至下腔静脉,达右心房平面,诊断为ICL。予体外循环腔静脉转流下腔静脉、左肾静脉平滑肌瘤切除、全子宫、双侧附件切除术治疗。患者手术顺利,术后恢复良好,出院后定期随访,无肿瘤复发表现。ICL需与子宫恶性肿瘤,深静脉血栓相鉴别。手术是治疗的主要方法,完整切除肿瘤是成功治疗的关键,术前要全面评估肿瘤范围。ICL预后好,有复发可能,需要长期随诊。Intravenous leiomyomatosis with intracardiac involvement is a rare disease with low diag-nostic rate before operation. The article reported a 52 year-old patient suffering from intravenous leiomyomatosis with intracardiac involvement enrolled in the First Affiliated Hospital of SUN Yat-sen University on 9 th May 2009. General PET-CT scanning showed multiple low density focus with irregular shape and undefined margin on uterine body,mainly located on the left side wall （4.0 cm ×2.0 cm ×3.2 cm）,and low density lesion in left ovarian vein,left renal vein and inferior vena cava. The patient was diagnosed as intravenous leiomyomato-sis with intracardiac involvement. The patient underwent myomatectomy （left renal vein and inferior vena ca-va）,hysterectomy,bilateral salping-oophenrectomy under the cardiopulmonary bypass. Successful operation was performed. The patient recovered well after operation and received regular follow-up. No episode of tumor recurrence was observed during the follow-up. The differential diagnosis includes malignant tumor and deep vein thrombosis. Operation is the main therapy and complete resection is the key. Total assessment for tumor extent is required before operation. Most patients have good prognosis but possible recurrence,so the long-term follow-up is needed.

关 键 词：血管内平滑肌瘤病 伴有心脏受累 诊断 治疗 

分 类 号：R737.33[医药卫生—肿瘤]