Liver and Vena Cava En Bloc Resection for an Invasive Leiomyosarcoma Causing Budd-Chiari Syndrome, Under Veno-Venous Bypass and Liver Hypothermic Perfusion : Liver Hypothermic Perfusion and Veno-Venous Bypass for Inferior Vena Cava Leiomyosarcoma.

BACKGROUND: Leiomyosarcoma of vascular origin is a rare tumor, occurring mainly in the inferior vena cava (IVC). When involving the hepatic vein confluence, it often causes Budd-Chiari syndrome, and IVC removal with a complex hepatectomy is required (Mingoli in J Am Coll Surg 211:145-146, 2010; Griffin in J Surg Oncol 34:53-60, 1987; Heaney in Ann Surg 163:237-241, 1966; Fortner in Ann Surg 180:644-652, 1974).

METHODS: A 57-year-old male, without previous oncological history, presented with Budd-Chiari syndrome due to a leiomyosarcoma extending to the supra-diaphragmatic IVC and involving the right and middle hepatic veins. The patient did not receive neoadjuvant treatment.

RESULTS: A femoral to superior vena cava veno-venous bypass was inserted, and both a median sternotomy and phreno-laparotomy with right subcostal extension were performed. A hemi-portocaval shunt was created between the right portal branch and the IVC, while a catheter was connected to the left portal branch for cold perfusion. Under extracorporeal circulation, the IVC was sectioned after infrahepatic and supra-diaphragmatic cross-clamping. The left liver was flushed with Celsior solution and packed with ice. A right trisectionectomy extended to the caudate lobe with en bloc vena cava removal was performed. The IVC was replaced by a cryopreserved aortic homograft, to which the stump of the left hepatic vein was anastomosed. Bypass duration, warm and cold liver ischemia, and operation time were 280 min, 8 min, 112 min, and 11 h, respectively. Duct-to-duct biliary anastomosis tutored by a T-tube was performed, and the patient was discharged on postoperative day 29, without major complications. After 16 months free of disease, the patient developed bilateral lung metastases. After 4 years the patient is still alive and receiving systemic chemotherapy.

CONCLUSIONS: Leiomyosarcoma of the IVC involving the hepatic veins can be treated with extended hepatectomy and removal of the IVC through extracorporeal circulation.