Introduction/Background Intravenous leiomyomatosis (IVL) can maliciously invade blood vessels and enter the systemic venous via the ovarian vein, or the parametrial uterine vein, or both. When the tumor extends into the iliac veins and the inferior vena cava (IVC), most patients present with lower-extremity edema and intravenous space-occupying lesions mimicking thrombosis. In extremely rare cases, IVL extending into the IVC can lead to Budd-Chiari syndrome (BCS).

Methodology We report the fourth patient with IVL who presented with BCS. Combined with a literature review of previously 3 reported cases, we summarized the clinical characteristics, treatment, and prognosis of secondary BCS caused by IVL.

Results A 49-year-old woman presented to our hospital with an 11-month history of bilateral leg edema accompanied by abdominal distension. Her surgical history included total abdominal hysterectomy (TAH) for a broad ligament myoma and a myomectomy of recurrent myoma. Abdominal ultrasound was significant for hepatomegaly with heterogeneous enhancement and occlusion of the hepatic venous reflux. Radiological imaging showed that the tumor originated from the pelvis, grew into the right common iliac vein and IVC, and finally invaded the right atrium (RA). Based on clinical symptoms and radiological evidence, a tentative diagnosis of BCS as a complication of IVL was established. The patient was managed with a one-stage operation involving intracanal tumor removal from the IVC and resection of the pelvic tumor, as well as bilateral adnexectomy and retroperitoneal lymphadenectomy.

Conclusion IVL can lead to secondary BCS, and its fatal complications may be avoided if handled properly and promptly. BCS caused by IVL should be considered when IVC and RA lesions occur in patients with characteristics of BCS and a history of uterine myoma or hysterectomy. Complete tumor resection with TAH plus bilateral salpingo-oophorectomy is the only reported curative treatment and should be performed as soon as possible.

Disclosures The authors have no conflicts of interest to declare.