子宫静脉内平滑肌瘤病的诊治进展

子宫静脉内平滑肌瘤病（intravenous leiomyomatosis,IVL）是一种罕见的疾病，可沿血管生长，延伸到下腔静脉，甚至心脏。目前对其发病机制尚有争议，多数学者考虑其起源于子宫肌瘤直接侵入子宫肌层静脉并沿血管腔扩散所致。子宫IVL好发于40～50岁有生育史的女性，早期临床表现不典型，如累及下腔静脉或右心时，可出现腹水、肝脾肿大、呼吸困难、心力衰竭，严重者甚至猝死。手术是目前首选的治疗方法，但具体的手术方案尚无统一共识。该病复发率高，术后需长期严密随访。综述子宫IVL的发病机制及高危因素、临床及影像学表现、诊断与鉴别诊断、治疗、预后及随访，以期为该病的诊疗提供参考。

Research Progress in Diagnosis and Treatment of Uterine Intravenous Leiomyomatosis

Uterine intravenous leiomyomatosis (IVL) is a rare disease. The tumor typically grows along vascular vessels and can extend to the inferior vena cava, even to the heart. The pathogenesis remains still controversial at present. Most scholars are inclined to consider that the leiomyoma directly invades into myometrial veins and spreads along the vascular lumen. Uterine IVL usually occurs in 40 to 50 years old women with a reproductive history. The clinical manifestations in the early stages are not typical. If the lesion involves the inferior vena cava or right heart, it can cause ascites, hepatosplenomegaly, dyspnea, heart failure, and even sudden death in severe cases. Surgery is currently the preferred treatment method, but there is no consensus on the specific surgical plan. The recurrence rate of the disease is high, and long-term and close follow-up is required after the operation. The pathogenesis, high risk fators, clinical and imaging manifestations, diagnosis and differential diagnosis, treatment and prognosis of uterine IVL are reviewed, in order to provide references for the diagnosis and treatment of the disease in the future.