A Case of Intravenous Leiomyomatosis with Involvement of a Renal Vein

Our patient is a 44-year-old woman who underwent exploratory laparotomy with total abdominal hysterectomy and bilateral salpingo-oophorectomy by gynecologic oncology for a suspected malignancy. The diagnosis of intravenous leiomyomatosis (IVL) was made instead. She was followed by her gynecologist and remained asymptomatic until 2 years later when she complained to the GYN of leg heaviness, and 2 months later also complained of palpitations. She was treated with a short course of megestrol without any clinical response. She was then referred to Vascular Surgery. A computed tomography scan of the abdomen and pelvis with intravenous contrast demonstrated intraluminal lesions in the inferior vena cava (IVC) extending to the mid-portion of the retrohepatic vena cava and into the left renal vein which could be followed to the blind end of the remnant of the left gonadal vein. Transabdominal resection was performed via a midline incision with reflection of the right colon and hepatic flexure to the left in conjunction with a Kocher maneuver and mobilization of the liver anteriorly, exposing the IVC from the bifurcation to the hepatic veins. Tumor inside the IVC could be palpated extending from the point of origin of the remnant of the right gonadal vein to below the level of the hepatic veins. The most superior portion of the tumor was mobile inside the IVC and was milked down to just above the renal veins. Control was obtained below and above the tumor as well as of the bilateral renal veins and lumbars. The patient was given systemic anticoagulation and after 3 min blood flow was interrupted. The IVC was opened via a longitudinal venotomy. The segment into the left renal vein could not be removed, and the left renal vein was amputated leaving a blind segment in the left renal vein without connection to the central venous circulation. The tumor originated inferiorly from the remnant of the right gonadal vein, and the venotomy was extended inferiorly to encircle the origin of the right gonadal vein. The remnant of the right gonadal vein was then resected en-masse with the tumor and a small island of IVC around it. The venotomy in the IVC was then closed. On follow-up, the patient is asymptomatic and without radiographic evidence of recurrence 22 months after surgery. On planning for resection of IVL, it is of critical importance to consider the proximal and distal extent of the tumor, but also the various routes through which the tumor may reach the central venous circulation, to include the bilateral hypogastric veins, the right gonadal vein, and the left renal vein via the left gonadal vein.