Leiomyosarcoma of the Inferior Vena Cava

Abstract

From 1979 to 2004, 22 patients were seen with leiomyosarcomas of the inferior vena cava (IVC). Twenty were treated surgically. Involvement of the IVC included the infrarenal segment in 3 cases, the suprarenal and/or retrohepatic segment in 13, and the suprahepatic segment in 4. Nineteen patients underwent wide tumor resection followed by ligation of the IVC in 5 cases, replacement with a PTFE prosthesis in 13, and cavoplasty in 1. An intracardiac tumor extension was resected during hypothermic circulatory arrest in 1 patient. Vascular exclusion of the liver was used in 5 cases and simple clamping of the IVC in 13 cases. There were 1 intraoperative death due to cardiac failure and 3 postoperative deaths due to multiple organ failure, liver failure, and duodenal fistula after treatment of a bleeding ulcer. Fifteen of the 16 surviving patients underwent adjuvant chemotherapy associated with radiation therapy in 4 cases. One patient was lost from follow-up at 10 months. Four patients including one with metastasis are still alive with a mean follow-up of 18.3 months. Eleven patients died after a mean follow-up period of 43.7 months due to local recurrence and/or distant metastasis in 9 cases and complications of chemotherapy in 2. The 3- and 5-year mean actuarial survival rates in patients who underwent resection were 52.0% and 34.8%, respectively. Leiomyosarcoma of the IVC is a serious disease. Although surgical resection combined with chemotherapy is usually not curative, it can achieve reasonably long-term survival. We recommend aggressive operative management using the latest vascular surgery and oncology techniques.

Resection and reconstruction of the inferior vena cava for neoplasms

“…Restoration of flow to the right renal vein by reimplantation (or pelvic kidney autotransplantation) is mandatory to maintain right kidney function, but optional for the left renal vein because of the left kidney's considerable collateral drainage through the adrenal, inferior phrenic, gonadal and paravertebral vessels [26] . Kieffer et al [19] used a proximal pressure reading of 30 mm Hg or more in the IVC as an indication for caval reconstruction and found reconstruction to be necessary in most cases. PTFE is the most commonly used prosthetic material and has been shown to be a suitable replacement for the IVC with excellent long-term patency [19,20,23,27,28] .…”

“…Kieffer et al [19] used a proximal pressure reading of 30 mm Hg or more in the IVC as an indication for caval reconstruction and found reconstruction to be necessary in most cases. PTFE is the most commonly used prosthetic material and has been shown to be a suitable replacement for the IVC with excellent long-term patency [19,20,23,27,28] . Infection and graft thrombosis are the 2 major complications of this type of reconstruction but both are rare.…”

“…If negative margins can be achieved, extended venous resection does not influence local recurrence rate or long-term outcome [21] . Radical resection of the tumor en bloc with the affected segment of the vena cava has been shown to be a feasible option with improved survival in multiple studies [17][18][19][20]22,23] . However, such patients have a poor prognosis and over half of them who undergo radical resection develop tumor recurrence; the 5-year survival rate ranges between 31% and 62% [24] .…”

“…However, even with extensive caval involvement, severe venous obstructive symptoms are not often seen, probably because of the development of extensive venous collaterals, which maintain adequate flow around the level of obstruction [17] . The segment of the IVC between the renal veins and the hepatic veins is the most commonly affected location for all primary vascular tumors [18][19][20] . Resection with negative margins is the treatment of choice [18] .…”

Sarcoma Resection With and Without Vascular Reconstruction: A Matched Case-Control Study

“…Over the last decade, several case series have established the feasibility and safety of en bloc vascular resection for sarcomas of the extremity, [3][4][5][6][7] retroperitoneum, [8][9][10] or specifically the inferior vena cava (IVC). [11][12][13][14][15][16][17][18] What remains unknown is whether these complex procedures are associated with a durable prolongation of survival that justifies their morbidity. Our institution has previously reported our findings with major blood vessel reconstruction on 14 sarcoma patients undergoing surgical resection.…”