Perioperative Management of Patient with IVC Leiomyosarcoma Resection: A Case Report

Inferior vena cava (IVC) leiomyosarcoma is a rare and aggressive malignant mesenchymal tumor that originates from the smooth muscle of the venous wall. First described by Perl in 1871, fewer than 400 cases have been documented globally to date [1]. These tumors typically exhibit slow growth, remaining asymptomatic for prolonged periods, and are often diagnosed incidentally. The clinical presentation of IVC leiomyosarcoma is typically delayed and nonspecific, with symptoms varying depending on the affected segment of the vein [2]. Diagnosis is primarily based on imaging studies, with confirmation achieved through biopsy [2]. Historically, involvement of the inferior vena cava has been considered a limiting factor for achieving curative resection in advanced cases [3]. Surgical resection of IVC leiomyosarcoma presents significant challenges, as it requires obtaining clear surgical margins and may necessitate vascular reconstruction. This can be accomplished using prosthetic or autologous grafts, primary suture, or, in some cases, simple ligation of the vena cava without reconstruction [2,4]. It is crucial to carefully assess which patients will benefit from IVC ligation as part of the surgical approach.

A 71-year-old female patient with a history of leiomyosarcoma involving the inferior vena cava (IVC) and extrahepatic infiltration. The tumor has extended above the renal veins into the hepatic hilum, reaching the confluence of the hepatic veins. Given the extent of the tumor, the patient was deemed a candidate for surgical intervention and subsequently admitted to the intensive care unit (ICU) for perioperative management.

Prior to the onset of the current condition, the patient had a history of grade 2 arterial hypertension, as classified by the ESC/ ESH guidelines, with a high cardiovascular risk profile. She also had a documented history of ventricular extrasystole and had undergone colon sigmoideum polypectomy as well as cholecystectomy. In relation to the present diagnosis, the patient underwent partial resection (debulking) of the IVC leiomyosarcoma followed by chemotherapy at another hospital. Her current medication regimen includes Propafenone, Ramipril, and Nadroparin (0.4 mL twice daily). The patient has no known allergies.

Surgery and peroperative management

Prior to the scheduled surgery, the patient had multiple invasive access lines in place, including a 3-lumen central venous catheter (CVC) via the left subclavian vein, a 2-lumen dialysis CVC via the right internal jugular vein, an epidural catheter, an arterial line, and a permanent urinary catheter. During the procedure for catheter placement, the patient experienced an accidental iatrogenic pneumothorax on the left side following subclavian vein puncture. This was managed with the insertion of chest drainage in the 6th intercostal space, with subsequent air suction from the pleural cavity. The patient then underwent surgery the following day under general anesthesia, after preoperative preparation that included 500 mg of methylprednisolone, a proton pump inhibitor (PPI), and premedication with Bromazepam. Anesthesia was administered using a combination of Sufentanil, Propofol, Sevoflurane, Ketamine, Atracurium, and epidural analgesia with Levobupivacaine and Sufentanil. The surgical procedure—resection of the suprarenal segment of the IVC with prosthetic replacement, right adrenalectomy, and resection of the first segment of the liverwas successfully performed.

Surgical Treatment Strategy

The surgical approach was determined based on preoperative CT imaging (Figure 1). Key factors influencing the decision included the involvement of the caudate lobe of the liver and the patency of blood flow within the affected segment of the inferior vena cava (IVC), specifically between the renal and hepatic veins, with only limited collateral circulation observed on imaging. Given these findings, resection without reconstruction was not considered viable. Instead, reconstruction using a ringed PTFE graft was planned. Considering the unpredictable perioperative hemodynamic consequences associated with IVC clamping and total vascular liver exclusion, a preparatory extracorporeal bypass from the infrarenal IVC to the subclavian vein was established, available for use if necessary. Following complete en bloc mobilization of the IVC segment involved by the tumor, the caudate lobe of the liver, and the right adrenal gland-both in close proximity to the tumor (Figure 2)-the IVC was clamped in the suprarenal segment below the tumor. The Pringle maneuver was then performed by clamping the hepatoduodenal ligament, including the portal vein and hepatic arteries, resulting in immediate hemodynamic changes. These changes were successfully managed with pharmacological interventions. As a result, we proceeded with resection without the need for the extracorporeal bypass. Resection and reconstruction were conducted utilizing a 20 mm ringed PTFE graft as an interposition bypass (Figures 3,4). The clamping duration was 40 minutes; following reconstruction of the proximal anastomosis just below the hepatic veins (lasting 15 minutes), blood flow to the liver was re-established, and the suprarenal anastomosis was completed subsequently.

Perioperative Management

Before IVC clamping, the patient received a heparin bolus at a dose of 100 units per kilogram. Continuous anticoagulation therapy with heparin was maintained in the postoperative period. The overall duration of the procedure was 265 minutes. To manage severe distributive shock during clamping, the patient required administration of a combination of vasopressors and catecholamines. Norepinephrine was administered at high doses, up to 4.00 mcg/kg/min, along with argipressin at 0.03 IU/min, angiotensin II at 40 ng/kg/min, and push-dose epinephrine (2x 100 mcg) to address hemodynamic instability. Additionally, Metoprolol 1 mg was administered to control tachycardia. Vasoactive drugs and fluids were titrated based on semi-invasive hemodynamic monitoring. Blood loss was carefully managed, with transfusions provided as necessary to maintain hemoglobin levels. Fluid balance included a total blood loss of 450 mL, 560 mL of blood products administered, 2550 mL of crystalloid fluids, and a diuresis of 730 mL. Blood glucose was closely monitored and managed with insulin. At the conclusion of the operation, the patient was transferred to the intensive care unit (ICU) under analgesosedation and mechanical ventilation. Circulatory stability progressively improved, allowing for a gradual reduction in the dose of catecholamines. Continuous heparin infusion was maintained due to the vascular reconstruction, and coagulation status (APTT) was monitored closely.

Postoperative management and treatment at ICU

Upon transfer to the ICU at 1:50 p.m., the patient was sedated, mechanically ventilated, and remained hemodynamically unstable. Bilateral pleural drainage was implemented: on the left side, active suction was applied with a negative pressure of -12 cm H₂O using a Thopaz device (without air leak), and on the right side, drainage was passive with a Heimlich valve. A postoperative chest X-ray confirmed bilateral pleural effusion without pneumothorax. Over the next several hours (9:00 p.m.), the patient’s circulatory instability deepened, necessitating an increase in the doses of norepinephrine, argipressin, and angiotensin II. Hemodynamic parameters were concerning, with a stroke volume (SV) of 15-25 mL, a cardiac index (CI) of 2.2, and a stroke volume variation (SVV) of 15-25%. A fluid bolus of 500 mL (composed of albumin and crystalloids) resulted in only minimal, transient improvement. Hemoglobin levels dropped appropriately, and an abdominal ultrasound revealed no active bleeding, with blood flow preserved. The patient experienced reduced diuresis, and echocardiography indicated left ventricular relaxation disorder along with moderate systolic dysfunction. The abdominal drains were outputting small amounts (2x70 mL) of sanguineous fluid. Laboratory results, including lactate, liver enzymes, acid-base balance, and glycemia, remained within acceptable limits. At 11:00 p.m., further deterioration of circulatory status prompted the addition of dobutamine to address impaired left ventricular kinetics. Sinus tachycardia was managed with Metoprolol. Due to persistent instability and oligoanuria, Levosimendan was introduced to augment cardiac function. Subsequent imaging confirmed intra-abdominal bleeding, leading to an emergency laparotomy approximately 12 hours after the initial surgery. After revision of the abdominal cavity, the patient’s hemodynamics stabilized, and the use of vasoactive agents was discontinued. Over the following hours, analgesosedation was gradually reduced, and the patient was sucessufully extubated with the initiation of spontaneous breathing approximately 30 hours post-revision. Post-extubation, the patient exhibited sufficient spontaneous respiration, and the chest drains were removed. Diuresis was fully restored, and there were no signs of infection, though the patient continued on antibiotic therapy. Nutritional support was initiated, and intensive rehabilitation, mobilization, and psychological support were commenced. Subsequently, while undergoing continuous anticoagulation therapy, the patient required two additional abdominal revisions due to continued bleeding, which occurred on the 4th and 8th postoperative days. Both procedures had an uneventful perioperative course. The patient was discharged home on the 22nd postoperative day, where she continues her recovery.

The long-term prognosis for patients with tumors invading the inferior vena cava (IVC) without curative resection is poor. The risk of IVC obstruction syndrome and recurrent pulmonary embolism significantly jeopardizes the patient’s life. Surgical resection of these tumors not only mitigates the risk of pulmonary embolism but also alleviates the clinical manifestations of hormonal hypersecretion and reduces the compression of surrounding organs caused by the tumor mass [5]. Total hepatic vascular exclusion (TVE), which involves clamping the portal triad as well as the IVC both above and below the liver, has proven beneficial for the resection of tumors involving both the IVC and the liver. This technique reduces intraoperative bleeding and the risk of air embolism [3]. A bloodless operative field facilitates precise dissection and reconstruction, contributing to a reduction in operation time. Additionally, in patients with chronic IVC obstruction, the development of collateral venous drainage through the ascending lumbar veins and the azygous/hemiazygous system helps mitigate hemodynamic instability during TVE, reducing the likelihood of needing cardiopulmonary or venovenous bypass [6]. However, rapid transfusion protocols and close anesthesiology support are crucial to maintaining stable hemodynamics during the procedure. Once hemodynamic status is stabilized, bypass is typically unnecessary; otherwise, venovenous bypass should be considered. IVC surgery presents significant challenges not only for the surgical team but also for the anesthesiology team. Manipulation of hepatic vessels and, particularly, clamping of the IVC are critical moments in the procedure. These maneuvers often result in compromised heart filling and reduced cardiac output, leading to severe distributive shock. To manage this, it is essential to administer a combination of vasoactive agents in high doses, alongside careful fluid management based on continuous hemodynamic monitoring. Ultimately, experienced management of severe shock allows successful completion of the procedure without the need for cardiopulmonary bypass.

Radical surgical resection remains the only potentially curative treatment for patients with leiomyosarcoma involving the inferior vena cava (IVC) [2]. Without resection, the median survival is limited to only one month [7]. Transdiaphragmatic intrapericardial IVC isolation is a simple and efficient technique that provides an option for patients who are not candidates for sternotomy or cardiopulmonary bypass (CPB) [3]. In conclusion, this technique appears to provide several advantages in the surgical treatment of IVC tumors, including the potential for a longer tumor-free margin, reduced operative time, and avoidance of sternotomy and CPB. However, further experience is required to fully validate this approach. From an anesthesiological perspective, managing the perioperative care of patients undergoing such procedures presents significant challenges. In the case we report, extreme circulatory instability was encountered during the isolation of the hepatic vessels and clamping of the IVC. The use of standard vasoactive agents (norepinephrine, argipressin) in unusually high doses, in combination with angiotensin II and targeted fluid management, effectively bridged the critical period of distributive shock without necessitating extracorporeal circulation or CPB. However, inadequate preparation, inappropriate patient selection, poor timing of surgery, and lack of experience in managing severe distributive shock can be fatal [8-11]. Therefore, we emphasize the importance of thorough preoperative preparation, clear communication, and strong teamwork as fundamental components for ensuring successful outcomes.

Prof. Daniel Pindak PhD. and surgical team of assistants, nurses, operating room staff.

Maria Michalovova MD., MPH and a team of fellow anesthesiologists, intensivists, nurses and ICU staff.

There is no conflict of interest.

1. Rusu CB, Corbatai L, Szatmary L, et al. (2020) Leiomyosarcoma of the inferior vena cava. Our experience and a review of the literature. Rom J Morphol Embryol 61(1): 227-233.
2. Castro FI, et al. (2023) Surgical resection of retrohepatic inferior vena cava leiomyosarcoma without vascular reconstruction: case report. J Vasc Bras 22: e20220108.
3. Chen TW, C H Tsai, S J Chou, C Y Yu, M L Shih, et al. (2007) Intrapericardial isolation of the inferior vena cava through a transdiaphragmatic pericardial window for tumor resection without sternotomy or thoracotomy. EJSO 33(2): 239-242.
4. Gaignard E, Damien Bergeat,Fabien Robin, Lisa Corbière, Michel Rayar, et al. (2020) Inferior vena cava leiomyosarcoma: what method of reconstruction for which type of resection? World J Surg 44(10): 3537-3544.
5. Chiche L, Dusset B, Kieffer E, Chapuis Y (2006) Adrenocortical carcinoma extending into the inferior vena cava: presentation of a 15-patient series and review of the literature. Surgery 139(1): 15-27.
6. Ciancio G, Soloway M (2005) Renal cell carcinoma with tumor thrombus extending above diaphragm: avoiding cardiopulmonary bypass. Urology 66(2): 266-270.
7. Mingoli A, Feldhasus RJ, Cavallaro A, Stipa S (1991) Leiomyosarcoma of the inferior vena cava: analysis and research of world literature on 141 patients and report of three new cases. J Vasc Surg 14(5): 688-699.
8. Anisuzzaman MD, Hosain M, Sarkar MR, Hallaz MM, Maruf MF, et al. (2023) Surgical management of inferior vena cava: A decade-long experience. Nigerian Journal of Cardiology pp. 91-96.
9. Kouz K, Thiele R, Michard F, et al. (2024) Haemodynamic monitoring during noncardiac surgery: past, present, and future. Journal of Clinical Monitoring and Computing 38(3): 565-580.
10. Li Shen, Lee MMY, Jhund PS, Granger Ch, Anand IS, et al. (2024) Revisiting Race and the Benefit of RAS Blockade in Heart Failure. Meta-Analysis of Randomized Clinical Trials. JAMA 331(24): 2094-2104.
11. Bruning R, Dykes H, Jones TW, Wayne NB, Newsome AS (2021) Beta-Adrenergic Blockade in Critical Illness. Front Pharmacol 12: 735-841.