Autotransplantation of the Liver for Ex Vivo Resection of Intrahepatic Caval Leiomyosarcoma: A Case Report

Intrahepatic caval leiomyosarcomas are rare tumors with limited therapeutic options as patients with the disease are not eligible for liver transplantation from the deceased-donor pool. Advances in surgical techniques gained in split and domino liver transplant procedures can be applied to resection of advanced tumors involving the hepatocaval confluence. Here, we describe the case of a 58-year-old white female who presented with visible abdominal wall collaterals and a palpable right subcostal tumor. Imaging revealed a 5.7 x 5.7 x 11-cm intrahepatic caval soft tissue mass extending into the hepatic veins, right renal vein, and infrarenal caval vein. The entire inferior caval vein was resected en bloc with the liver and right kidney and replaced with a blood group-identical fresh caval vein graft from a deceased donor. The splanchnic circulation was decompressed with a temporary portocaval shunt to the caval vein graft, and caudal inflow into the caval vein graft was established with a left iliac anastomosis. Ex vivo resection of the native inferior caval vein containing the intravascular tumor together with a sleeve of liver was performed under hypothermic conditions, and hepatic outflow was reconstructed with vein from the deceased donor. The liver was autotransplanted via the classical piggyback technique with uneventful portal reperfusion following a cold ischemic time of 2 hours. Histology confirmed a grade 3 leiomyosarcoma with clear resection margins. Liver function was stable, and the patient is currently alive at 2 years after resection. Follow-up imaging at 12 months was unremarkable, but local recurrence was detected on the most recent computed tomography scan. In conclusion, ex vivo resection of an intrahepatic caval leiomyosarcoma with inferior caval vein replacement by a deceased-donor caval graft and subsequent liver autotransplantation are technically demanding but provide a chance on prolonged survival.