Liver Autotransplantation for an Inferior Vena Cava Tumor

Inferior vena cava (IVC) tumors are rare and have been reported to account for approximately 0.5% of all sarcomas affecting adults and represent fewer than one in 100,000 of all malignancies (1). The only possible treatment is, generally, resection. There have been several reports which showed the successful removal of an IVC leiomyosarcoma with the infrahepatic IVC using artificial vessels (2–4). However, there have been a few reports of successful removal of a far advanced IVC tumor, extended beyond the orifice of the hepatic veins (5, 6). We herein describe the first case of an IVC tumor that had extended to the right atrium, which was successfully treated with extracorporeal resection and liver autotransplantation. A 40-year-old woman presented with leg edema and was eventually diagnosed to have an IVC tumor originating at the level of infrahepatic IVC, extending to the right atrium (Fig. 1A, B). After an upper midline abdominal incision was made, the whole abdomen was found to be free of peritoneal dissemination. Subsequently, the incision was extended to a long thoracoabdominal incision with midline sternotomy. After Kocher duodenal mobilization, the IVC tumor was found to be resectable without the need for pancreaticoduodenectomy. However, the tumor extending to the right atrium could not be dissected from the wall of the IVC at the level of the hepatic veins. Because three confluences of the major hepatic veins were involved, the liver and IVC, including the whole tumor, needed to be removed en bloc with a cardiotomy. Under extracorporeal circulation from the portal vein, the infrarenal IVC and the superior VC to the ascending aorta with an artificial cardiopulmonary device, the liver with the IVC and PV, HA, and common bile duct were removed en bloc.FIGURE 1: A, Contrast-enhanced computed tomography showed an IVC tumor (arrow). B, Schematic drawing of the tumor originating at the level of infrahepatic IVC, extending to the right atrium. C, The IVC tumor was separated from the liver on the back table. IVC, inferior vena cava.In a basin with ice-cold histidine-tryptophan-ketoglutarate solution, the IVC, including the tumor, was resected (Fig. 1C). Subsequently, the liver was flushed by the portal vein with cold histidine-tryptophan-ketoglutarate solution. The hepatic veins were anastomosed to the prosthetic polytetrafluoroethylene (PTFE) graft (Goretex, W. L. Gore and Associates Inc., Newark, DE), which was 20 mm in diameter in an end-to-end manner (Fig. 2A–C) to ensure the outflow of the liver. First, the rings of the PTFE graft at the estimated anastomosis sites were removed, and the longitudinal hole for the right hepatic vein was made on the PTFE graft. The anastomosis was made with 4-0 nonabsorbable Polypropylene (Prolene) running sutures followed by making the transverse hole for the middle or left hepatic vein to allow for better adjustment of the location in the same manner. Liver autotransplantation with prosthetic IVC was performed using the same procedure used for conventional whole-liver transplantation. Because the caudal tip of the IVC tumor was located at the level of confluence of the right renal vein (RRV), we temporarily clamped the RRV and cut it at the confluent to IVC. Subsequently, the RRV was anastomosed to the caudal side of the remaining native IVC. Therefore, the right kidney was not removed, whereas the left renal vein was completely transected. The duration of the entire operation was 859 min. The blood loss was 15,500 cm3, including the blood that was used to flush the liver and IVC using an autologous blood recovery system. The postoperative course was uneventful in the intensive care unit, without any significant derangement in the liver function. The pathologic diagnosis indicated leiomyosarcoma of the IVC. The patient is currently doing well with good liver function and patent hepatic veins, as confirmed by contrast-enhanced computed tomography (Fig. 2D) at 6 months after the surgery. There have been several reports of aggressive hepatectomy using a back table procedure for advanced liver tumors, including those using liver transplantation techniques (7, 8). We herein showed that such an approach can be applied not only for liver tumors but also for advanced IVC tumors. When the tumor is located at the infrahepatic IVC, it can be removed while preserving the liver. When the tumor extends to the level of the orifice of the hepatic veins, IVC tumor removal with right or left hepatectomy can be performed (9). However, in the present case, the tumor extended beyond the hepatic veins up to the right atrium, so we decided to perform liver autotransplantation with removal of the IVC tumor with the whole liver.FIGURE 2: A, The right hepatic vein was anastomosed to the prosthetic IVC. B, The trunk of the middle and left hepatic vein was ready to be anastomosed to the prosthetic IVC. C, Just before implantation back to the patient. D, Computed tomography showed the patent hepatic veins after liver autotransplantation. IVC, inferior vena cava.Liver autotransplantation has been reported after extracorporeal liver resection; however, there have been no reports of the treatment of an IVC tumor using this technique. Another option to resect the IVC tumor could be in situ excision with cold preservation through the portal vein with the liver in place, called hypothermic preservation (10, 11). However, it was thought that it would be difficult to reconstruct all three hepatic veins with the prosthetic IVC in situ because the liver cannot be flipped back in situ to make a precise anastomosis, unless a hemihepatectomy is performed in cases with severely congested liver. There have been several reports indicating that only resection may offer a chance of long-term survival for patients suffering from leiomyosarcoma of the IVC (12). We have not considered using any adjuvant chemotherapy because of the lack of evidence of any residual tumor so far. However, a close follow-up is needed because of the malignant nature of the tumor. In conclusion, we experienced a rare case of IVC leiomyosarcoma extending to the right atrium, for which extracorporeal resection, followed by liver autotransplantation with a PTFE graft substituted for the original IVC. Only the liver transplant technique allowed us to complete the total excision of the tumor to potentially provide long-term survival. Therefore, close collaboration between transplant and Hepatopancreatic-biliary surgeons can make this type of complicated surgery possible. Mitsuhisa Takatsuki 1 Susumu Eguchi1,2 Koji Hashizume1 Akihiko Soyama1 Masaaki Hidaka1 Kazuyoshi Tanigawa1 Kiyoyuki Eishi1 Tamotsu Kuroki1 1 Department of Surgery and Cardiovascular Surgery, Nagasaki University Graduate School of Biomedical Sciences Nagasaki, Japan