累及右侧心腔的静脉平滑肌瘤病罕见并具有潜在致命性、激素依赖性及沿血管腔蔓延的特点，以手术治疗和内分泌治疗为基础的多学科综合治疗是最佳疗法并有望改善预后。文章报道1例累及右心房的静脉平滑肌瘤病病例，经心脏超声、胸部和腹部CT、CT血管造影、病理学检查明确诊断，予三苯氧胺联合诺雷德内分泌治疗并行分期手术；一期行心脏及下腔静脉肿瘤切除术获成功。

Intravenous leiomyomatosis with right atrium extension is a rare tumor with potential lethality, hormonal dependency and vascular extension. Surgical excision and hormonal therapy-based multi-discipline cooperation therapeutics may be the optimal therapy and helpful to improve the prognosis. One case of intravenous leiomyomatosis with right atrium extension which was diagnosed by examinations of echocardiography, chest and abdomen CT, CT angiography and pathology was reported in this paper. Tamoxifen and luteinising hormone-releasing hormone agonist were administered as endocrine therapy, and the tumor was resected through a two-stage approach. The patient underwent one-stage resection for the mass of right atrium and inferior vena cava, and the outcome was satisfactory.