IntroductionLeiomyosarcoma emanating from the vascular wall of the inferior vena cava (IVC) is rare. Although most IVC tumors are amenable to operative procedures, such as either IVC ligation or wedge resection of the IVC with simple closure or patch reconstruction rather than prosthetic replacement, 1,2 it is not certain which surgical procedure is better when a long segment of the IVC and one kidney are involved. We herein report a patient with a primary leiomyosarcoma of the IVC who successfully underwent resection of the affected IVC and right kidney along with graft replacement of the IVC.
Case historyA 48-year-old Chinese woman was admitted with more than 3 months' history of mild pain in the right flank and right epigastric region before seeking medical attention. Prior to then she had always been healthy. Physical examination revealed a large tumor in the right flank and right mid-abdomen. Abdominal ultrasonography demonstrated a large retroperitoneal mass (17 cm ϫ 14 cm ϫ 8 cm) surrounding the right kidney in the right epigastric region. Magnetic resonance imaging (MRI) and computed tomography (CT) revealed a tumor that originated from the IVC wall at the confluence of the renal vein with the IVC.The right kidney was included in the tumor. MRI also showed that the mass was 16 cm in longitudinal diameter and 6 cm in width in the corresponding portion of the IVC ( Figure 1A). There was no obvious IVC thrombus identified around the tumor. Intravenous urography indicated that the function of the left kidney was normal but that the right kidney was nonfunctional. Blood urea nitrogen and serum creatinine values were normal.The patient was taken to the operating room 10 days later. The abdomen was entered through an upper midline incision. Exploration of the abdomen confirmed the preoperative findings. The tumor involved the infrarenal and suprarenal IVC, as well as the right kidney, but there were no other metastases present. The infrarenal and suprarenal IVC and left renal veins together with the right renal artery were controlled and dissected. Several lumbar veins were also ligated and divided to free the IVC approximately 2 cm above and below the tumor to allow for an adequate margin of resection. The IVC was clamped proximally just below the liver and distally just above its bifurcation, and the distal left renal vein was also clamped. The IVC tumor and the involved right kidney were removed, leaving 1 cm of IVC or left renal vein cuffs for anastomoses. The IVC was replaced in an end-to-end fashion with a polytetrafluoroethylene graft, 16 cm in length, and the distal left renal vein was anastomosed end-to-side to the middle one third of the graft. After completion of all three anastomoses, the clamps were removed. During this period, the hemodynamics were stable and the left kidney showed no obvious signs of congestion. The entire operative time was 3 h and 45 min and the blood loss was 1200 ml.The resected IVC specimen showed tumor protrusion into the lumen of the IVC and the right renal vein. Histopath...