Extended right hepatectomy and VCI resection to achieve R0 resection of a VCI leiomyosarcoma

Abstract

Background: Leiomyosarcoma (LMS) of the vena cava inferior (VCI) is an extremely rare disease. The only curative treatment is achieved by R0 resection of the tumor. Methods: Here we present a case of a 10x8x8cm LMS of the VCI that was successfully R0 resected with extended right hepatectomy and enbloc VCI resection. Preoperative right portal vein embolization was performed to enlarge the future liver remnant. Complete VCI resection was performed transdiaphragmatically with clamping of the right atrium. The reconstruction was made with reimplantation of the left hepatic vein, two renal veins and right iliac vein on a 20mm goretex graft. Biliary reconstruction was made with Roux-Y cholangiojejunostomy with individual anastomosis of the Segment 2 and 3 bile ducts. Results: Postoperative course was eventless and the patient is tumor free 6 months postoperatively. Conclusion: R0 resection of VCI LMS can be achieved in tertiary HPB centers with capacity of multidisciplinary approach.