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

Abstract

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.