Ex Vivo Liver Resection and Autotransplantation as Surgical Option for Zone II–III Leiomyosarcoma of IVC: A Case Report and Literature Review

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.

Successful Right Hepatectomy for Recurrent Liver Tumor Originating from an Inferior Vena Cava Leiomyosarcoma: A Follow-Up Case Report

“…The rates of successful radical resection or survival appear to be related to tumor extension zones [ 9 ]. According to previous reports [ 10 – 12 ], in cases of tumor extension that spread to the confluences or tributaries of the hepatic veins, liver autotransplantation was necessary for IVC-LMS in zones II–III, and survival rates of over 1 year could be achieved. In the initial operation in the present patient, the tumor tip extended to the orifice of the hepatic vein trunk, and curettage of the floating piece of the tumor was performed by the cardiologist by opening the transected stump of the IVC.…”

Surgical management strategy for leiomyosarcoma of Zone I-II inferior vena cava

“…The reported cumulative 5-year overall survival (OS) rate and 5-year disease-free survival (DFS) were 55% and 5% respectively [5,17,12,18] . Postoperative recurrence does not preclude re-resection of the leiomyosarcoma [3] . No randomized clinical trial evaluates the clinical efficacy of neoadjuvant chemo- or radiotherapy.…”

“…The remaining one patient was II-III leiomyosarcoma and underwent ex vivo Liver resection and autotransplantation with atrial reconstruction. [ 3 ] The detailed baseline information and clinical characteristics of these four patients are given in Table 1 .…”

“…During the period, five patients with IVC leiomyosarcoma were treated in our center, among them, four patients were categorized into zone I-II leiomyosarcoma and underwent radical resection. The remaining one patient was II-III leiomyosarcoma and underwent ex vivo Liver resection and autotransplantation with atrial reconstruction [3] . The detailed baseline information and clinical characteristics of these four patients are given in Table 1.…”

“…Primary leiomyosarcomas is originated from inferior vena cava (IVC) wall and presents as a very rare clinical entity [2] . It grows slow and insidiously without significant symptoms at early stage; thus, it is extensively advanced with multi-organ and vascular involvement when the diagnosis of leiomyosarcoma is confirmed [3,4] . With the advancement of surgical oncology and its adjuncts, radical resection has become the only curative option considering poor clinical efficacy of chemo and radio-therapy [5] .…”