Leiomyosarcoma of the inferior vena cava.

Leiomyosarcoma of the inferior vena cava (IVC) is a rare malignancy. Approximately 350 cases of this disease are known worldwide. Surgical treatment of this condition remains an extremely difficult task. In this case report, we present an experience of successful radical treatment of a leiomyosarcoma of the suprarenal IVC without vascular reconstruction. The patient was treated with resection of the suprarenal portion of the IVC with the tumor; however, in view of adequate blood outflow from the right lobe of the liver, prosthetic replacement of the IVC with right hepatic vein reimplantation was not performed.

Leiomyosarcoma of the inferior vena cava (IVC) is a rare malignancy. Radical surgery is the main treatment option and allows to achieve 5- and 10-year survival rates of 49.4% and 29.5%, respectively., Here we present a case report of a patient with IVC leiomyosarcoma.

Consent for publication was obtained from the patient.

Case report

A 65-year-old woman was admitted to the A.V. Vishnevsky National Medical Research Center of Surgery complaining of dyspnea and leg swelling. Abdominal multislice computed tomography (MSCT) with intravenous contrast enhancement demonstrated a tumor measuring 78 × 43 × 55 mm in the suprarenal IVC, on the outer wall of the left semicircle. The tumor invades the IVC wall occluding it from the ostium of the left renal vein to the diaphragm (Fig 1, A). Magnetic resonance imaging of the abdomen revealed a mass lesion in the projection of the IVC at the hepatic segment with a blood clot in the lumen (Fig 1, B). The left hepatic vein is not involved in the tumor process (Fig 1, C). Fig 1, D, shows the preoperative MSCT of the patient, where functioning cava-caval anastomoses in the retroperitoneal space are visible.

Fig 1

A, Abdominal and retroperitoneal multislice computed tomography (MSCT) (the arrow indicates the inferior vena cava [IVC] tumor). B, Abdominal and retroperitoneal magnetic resonance imaging (the arrow indicates IVC tumor). C, Abdominal and retroperitoneal MSCT: (1) intact left hepatic vein; (2) tumor. D,Arrows show cavacaval anastomoses in the retroperitoneal space.

The patient underwent surgery in March 2019. Resection of the IVC with the tumor and subsequent prosthetic replacement with right hepatic vein reimplantation were planned.

After initiating endotracheal anesthesia, the abdominal cavity was opened using a J-shaped incision. Visual inspection revealed a neoplasm originating from the IVC: dense elastic, with an irregular round shape, located on the anterior left wall of the IVC wall. The tumor was adjacent to the posterior surface of the liver. The tumor measured approximately 10 × 7 × 6 cm. The lower pole of the tumor was located at the level of the ostium of the right and left renal veins in the IVC. The upper pole of the tumor was located at the level of the ostium of the right hepatic vein in the IVC. The right hepatic vein was involved in the tumor.

The hepatoduodenal ligament was clamped twice during the separation of the tumor and mobilization of the liver: for 6 minutes and for 3 minutes for ischemic preconditioning of the liver. Complete vascular isolation of the liver was achieved (the infrarenal IVC, suprahepatic IVC, and hepatoduodenal ligament were clamped). The right hepatic vein was clamped and resected. The suprarenal IVC with the tumor was resected. The clamp was transferred from the suprahepatic IVC to below the ostium of the left hepatic vein. The clamp on the hepatoduodenal ligament was removed. The liver isolation time was 7 minutes.

Intraoperative ultrasound examination was carried out, revealing a patent right renal vein in the renal hilum and veins with a blood flow in the kidney's periphery. The middle hepatic vein was patent. Venous shunts were observed in liver segments IV and VI and in the left lobe. The portal vein was patent in color flow scanning mode. In view of the absence of signs of severe liver edema with the right hepatic vein being clamped, the venous shunts from the right hepatic vein territory to the left hepatic vein territory, adequate blood outflow from the right lobe of the liver as demonstrated on intraoperative ultrasound examination, the absence of marked hemodynamic disturbances after the clamping and resection of the IVC, and the preoperative evidence of thrombosis of the suprarenal IVC, a decision was made to abstain from prosthetic replacement of the IVC with right hepatic vein reimplantation. The proximal end of the IVC was sutured immediately under the ostium of the left hepatic vein. The trunk of the right hepatic vein was sutured in the hepatic parenchyma. The distal segment of the IVC was sutured above the ostia of the right and left renal veins. Intraoperative photographs and a tissue specimen are presented in Fig 2.

Fig 2

A, Intraoperative photograph. (1) Infrahepatic segment of the inferior vena cava (IVC) on a holder. (2) Tumor. B, Intraoperative photograph of the surgical field after resection of the inferior vena cava (IVC) with the tumor. Arrows show IVC stumps. C, Resected IVC segment with the tumor. D, Tumor specimen, cross-section of the tumor.

In the postoperative period, the patient received anticoagulant therapy to prevent thromboembolic complications (enoxaparin sodium 0.4 daily). We evaluated the liver function in the preoperative and postoperative periods. In this case, there was a moderate increase in transaminases (alanine aminotransferase, 127 U/L; aspartate aminotransferase, 111 U/L) and alkaline phosphatase (678 U/L) in the postoperative period.

Histology report

Leiomyosarcoma of the IVC, grade 2 (5 of 8 according to the National Federation of French Cancer Centers grading system: moderate differentiation, 2 points; number of mitoses, 2; tumor necrosis percentage (<50%), 1 point; dimensions, 9.5 × 6.5 × 5 cm. Fig 2, D, presents a cross-section of the tumor.

The postoperative period was complicated by acute renal failure, which necessitated temporary renal replacement therapy. This condition resolved on postoperative day 5.

Duplex scanning (on postoperative day 13) of the liver revealed patent portal and left and middle hepatic veins with fully colored lumina status post resection of the right hepatic vein. Collateral blood flow in the right lobe of the liver is of note. The renal veins are patent in the hila of both kidneys. MSCT (on postoperative day 13) demonstrated thrombotic material and isolated, small areas of poor contrast enhancement along the entire length of the lumina of the IVC, iliac veins, and partially visualized lower limb veins (Fig 3). Collateral veins (cavocaval shunts) were observed in the retroperitoneal and abdominal adipose tissue, as well as in the subcutaneous fat. The patient was discharged on postoperative day 17.

Fig 3

Abdominal and retroperitoneal multislice computed tomography (MSCT) after tumor resection. Inferior vena cava (IVC) stumps are shown by white arrows.

During the follow-up examination 9 months after the operation, the patient does not show complaints. Duplex scanning showed the left and middle hepatic veins to be passable, monophasic blood flow is recorded in the IVC below the inflow of the renal veins. There is no evidence of tumor recurrence.

Discussion

There is currently no conventional algorithm for surgical treatment of this tumor, because types of surgical intervention and the need for IVC reconstruction after resection are considered on an individual basis. The adequacy of collateral venous blood flow resulting from lasting IVC occlusion is the decisive factor for the choice of surgical treatments.

Slow tumor growth usually promotes the development of a collateral network, which subsequently allows radical surgery without vascular reconstruction. According to literature data, such situations are usually encountered in patients with infrarenal IVC involvement. IVC ligation is associated with shorter operative time and a lower risk of postoperative complications, such as pulmonary embolism.

The advantage of IVC prosthetic replacement is prevention of leg swelling and acute renal failure. The reconstruction of the IVC with a prosthesis is more optimal in most cases; however, in our particular case, we were guided by the severity of the patient's somatic status in the choice of surgical tactics; collegially it was decided that it is acceptable.

In conclusion, we would like to underline the essential role of intraoperative duplex ultrasound examination in the assessment of venous blood flow in the hepatic parenchyma, which has implications for further surgical decision making. IN addition, visual examination of the state of the liver is decisive, because it can demonstrate the absence of hepatic parenchyma edema resulting from venous congestion, as well as the color and structure of the parenchyma.

In view of the low prevalence of this disease, each case report described in the literature is informative.