静脉内平滑肌瘤病(IVL)是一种少见、特殊类型的子宫平滑肌瘤,其组织病理学为良性病变,生长方式类似恶性肿瘤,可在静脉血管内生长,但很少浸润组织。肿瘤沿静脉回流方向生长,通过子宫静脉、卵巢静脉超出子宫范围延伸至下腔静脉内,直至长入右心房或肺动脉而累及心脏,为心脏内平滑肌瘤病(ICL)。目前超声波、电子计算机体层扫描(CT)、磁共振成像(MRI)均可作为检测肿瘤大小和范围的有效方法。治疗ICL以手术为首选,手术方式包括一期手术和分期手术,彻底切除肿瘤是治疗的关键。大多数患者手术后效果较好,但亦有肿瘤复发的可能。由于该病罕见,发病隐匿,临床表现多样,缺乏特异性,且具有潜在的致命性,导致临床工作中易出现漏诊、误诊。对ICL的研究和诊疗进展进行综述,以提高临床医生对该病的认识。 Intravenous leiomyosarcoma (IVL) is a rare, special type of uterine leiomyoma whose histopathology is a benign lesion that grows like a malignant tumor and grows in the vein but rarely infiltrating the tissue. The tumor grows along the direction of venous return and extends through the uterus to the inferior vena cava through the uterine vein and into the inferior vena cava until it grows into the right atrium or pulmonary artery involving the heart, which is intracardiac leiomyomata (ICL). At present, ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI) are both effective methods to detect tumor size and range. The treatment of ICL to surgery as the preferred method of operation, including a surgical stage and staging surgery, complete removal of the tumor is the key to treatment. Most patients are better after surgery, but also the possibility of tumor recurrence. Because of the rare disease, occult onset, diverse clinical manifestations, lack of specificity, and potentially fatal, leading to clinical misdiagnosis, misdiagnosis. The progress of research and diagnosis and treatment of ICL is reviewed in order to improve clinicians’ understanding of the disease.