Abstract
We report the first documented case of leiomyosarcoma at zone II-III of inferior vena cava with thrombi in three hepatic veins undergoing ex vivo liver resection and autotransplantation (ELRA) and hepatic veins thrombectomy. A 33-year-old female patient presented with abdominal distention and lower extremities edema. Abdominal wall varicosis and shifting dullness were positive on physical examination. Her liver function was classified as Child-Pugh B and a solid tumor at retro-hepatic vena cava extending to right atrium with thrombi in three hepatic veins were confirmed. The diagnosis of leiomyosarcoma with Budd-Chiari syndrome was highly suspected with preoperative ultrasound, echocardiogram, CT scan, and three-dimensional reconstruction. A zone II-III leiomyosarcoma of IVC origin was confirmed at surgery and ex vivo liver resection and autotransplantation, and hepatic vein thrombectomy with atrial reconstruction were performed under cardiopulmonary bypass (CPB). Operative time, anhepatic time, and CPB time were 12 h, 128 min, and 84 min, respectively. The patients experienced post-operative liver dysfunction and was cured with conservative therapy. Hepatic recurrence two years after surgery was managed with radiofrequency. The patient was alive with liver metastasis three years after surgery. Despite being regarded as an extremely aggressive procedure, ELRA could be considered in the treatment of advanced leiomyosarcoma with Budd-Chiari syndrome and hepatic vein thrombi.
Highlights
Leiomyosarcoma is a rare malignant tumor originating from the smooth muscle, with a small subset of vascular origin, especially from inferior vena cava (IVC)
Primary leiomyosarcomas of IVC originate from the smooth muscle of vascular wall and present as a rare clinical entity [8]
They usually grow slowly and asymptomatically and, at the time of diagnosis, become very large in size and extend into renal, hepatic veins, and atrium resulting in compromised liver and renal function, making curative surgical resection difficult [9]
Summary
Leiomyosarcoma is a rare malignant tumor originating from the smooth muscle, with a small subset of vascular origin, especially from IVC. ELRA with bench thrombectomy was planned considering tumor involvement to three hepatic veins and right atrium as well as thrombi. The hepatic inflow and outflow were disconnected for explanting the whole liver with the tumor (Figures 2A, B) At this time, two independent teams performed bench resection and maintained hemodynamic stability, respectively. Anterior wall of IVC and hepatic veins’ orifices without tumor; resection margin was confirmed by repeated frozen section. Temporary porto-systemic shunt was discontinued, and portal vein, hepatic artery, and common bile duct were anastomosed in end-to-end fashion By this time, the clamps were removed and autograft was perfused (Figure 2F). Postoperative ultrasound showed patent hepatic veins, portal vein, hepatic artery, and IVC She was discharged at postoperative day 21 with normal liver and heart function with oral diuretics. She is 32 months after surgery with regular follow-up
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