Leiomyosarcoma of the Retrohepatic Vena Cava Treated by Excision and Reconstruction With an Aortic Homograft: A Case Report and Review of Literature

Abstract

Leiomyosarcoma of the retrohepatic inferior vena cava is a rare entity and presents a number of diagnostic and therapeutic challenges. Here such a case is presented in which the retrohepatic inferior vena cava was excised after full mobilization of the liver under venovenous bypass. The continuity of the vena cava was restored with cryopreserved aortic homograft. The technical details with regard to total vena caval clamping, venovenous bypass, hepatic inflow occlusion, techniques of reconstruction, including the use of cryopreserved aortic homograft, and a brief review of the literature on the surgical management of retrohepatic inferior vena caval tumors are discussed.

Surgical Challenges in the Treatment of Leiomyosarcoma of the Inferior Vena Cava: Analysis of Two Cases and Brief Review of the Literature

“…However, there is a risk of significant complications, such as venous insufficiency of the lower limbs, resulting in oedema, deep vein thrombosis, ascites, and renal dysfunction. 2,4,7 Kieffer et al used a proximal pressure in the IVC of 30 mm Hg or more as an indication for caval reconstruction, showing that reconstruction seems to be necessary on most cases. 12 When dealing with tumors of the inter-or suprarenal segment of the IVC, reconstruction can be achieved by prosthetic graft and reimplantation of the renal vein(s).…”

“…Autotransplantation of the right kidney into the right iliac fossa can be performed, after prosthetic reconstruction of the IVC. 2,5,7,11 Otherwise, right nephrectomy might be required for tumors of segment II of the IVC, even if the kidney is not directly involved. If the tumor involves the infrarenal IVC, surgical options also include simple ligation or graft reconstruction.…”

“…The fact that almost 10.5% of these tumors are discovered incidentally and up to 33% are autopsy findings is highly typical. 7,11 The most common presenting symptom of these tumors is pain in the right flank, which is often long-standing and can mislead diagnosis. Other nonspecific symptoms, such as anorexia and vomiting, can occur from the masseffect of the tumor.…”

“…7 The accumulated experience in the literature up to date results mainly from scattered casereports, small series, and an effort for an international registry, published in 1996. 1,5 Only 2% of leiomyosarcomas are of vascular origin, but its most typical vascular location is in the IVC, representing approximately 50% of the cases.…”

Surgical Management of Leiomyosarcoma of the Inferior Vena Cava

“…Although it is not an absolute contraindication, invasion of the aorta by the tumor requires additional major vascular reconstructive procedures, and it should be carefully considered on pre-operative imaging studies. 9,12,18,19 The presence of Budd-Chiari syndrome portends an advanced disease in that the cause of death among the patients reported in the literature with unresectable IVC leiomyosarcoma was Budd-Chiari syndrome in two thirds of cases. 1 Therefore, involvement of the hepatic veins by the tumor is an important variable determining feasibility of curative resection, and its poor prognosis should be kept in mind before offering surgical resection.…”