A 2-Stage Surgical Approach for Adrenocortical Carcinoma With Intracardiac Extension
Pietro Addeo, MD, Jean-Philippe Mazzucotelli, MD, PhD, and Philippe Bachellier, MD
Department of Hepato-Pancreato-Biliary Surgery and Liver Transplantation, Hautepierre Hospital, University Hospitals of Strasbourg, University of Strasbourg, Strasbourg, France; and Department of Cardiothoracic Surgery, Nouvel Hôpital Civil, University Hospitals of Strasbourg, University of Strasbourg, Strasbourg, France
Direct intracardiac extension of abdominal malignant diseases represents a rare but challenging situation. Removal of the intracardiac extension requires car- diopulmonary bypass with systemic anticoagulation, which could potentially increase the risk of bleeding if it is associated with liver resection. This report describes a 2-stage surgical approach for malignant disease with intracardiac extension in a high-risk patient. Atrial thrombectomy was performed first, followed by right portal vein embolization. Four months after the cardiac surgical procedure, the patient underwent right hepa- tectomy extended to segment 1, the right adrenal gland, and the retrohepatic inferior vena cava under venovenous bypass. The advantages and drawbacks of this approach are discussed.
(Ann Thorac Surg 2022;114:e371-e373) @ 2022 by The Society of Thoracic Surgeons
D irect intracardiac extension of abdominal ma- lignancies represents a rare but challenging sit- uation. Renal cancer, adrenocortical carcinoma, and hepatocellular carcinoma can spread directly as thrombotic masses through the inferior vena cava up to the right atrium. Atresia of the tricuspid valve caused by direct cardiac extension can lead to sudden death and represents a surgical emergency. En bloc resection of the primary tumor with tumor thrombus is commonly indicated in fit patients without distant metastases.1,2
Removal of the intracardiac extension requires car- diopulmonary bypass with systemic anticoagulation, which could potentially increase the risk for bleeding if it is associated with liver resection. In addition, major liver resection can necessitate patient preparation by portal
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vein embolization in the presence of a small volume of the future liver remnant. Thus, hypertrophy of the future liver remnant requires at least 4 weeks to develop. We present a case of a patient with adreno- cortical carcinoma with tumoral thrombosis extending through the inferior vena cava into the right atrium, for which we chose to perform cardiac surgery and liver resection as a 2-stage procedure.
In April 2020, a 63-year-old man was referred to the Department of Hepato-Pancreato-Biliary Surgery and Liver Transplantation, Hautepierre Hospital, University Hospitals of Strasbourg for the treatment of a 17-cm- diameter adrenocortical carcinoma with macrovascular invasion (tumoral thrombus), which extended through the right adrenal vein into the retrohepatic inferior vena cava up to the right atrium (Figure 1). The lesion was discovered during a diagnostic workup for lumbar pain in March 2020. The patient had had a nose swab that tested positive for COVID-19 and typical pulmonary chest computed tomo- graphic images, but he had few symptoms.
Given the radiologic presentation, we decided to perform combined abdominal (right hepatectomy and segment 1 with right adrenalectomy) and intracardiac thrombectomy under cardiopulmonary bypass. Preoper- ative coronary angiography showed 2 coronary stenoses requiring bypass grafts. Preoperative anticoagulation was started, and the patient presented with severe thrombo- cytopenia. A serologic test revealed heparin-induced thrombocytopenia. Therefore, bivalirudin was chosen for anticoagulant therapy for cardiopulmonary bypass.
A 2-stage surgical removal of the tumor was chosen because of (1) the potential for increased bleeding during liver resection under cardiopulmonary bypass, (2) the need for right liver hypertrophy before major hepatec- tomy, (3) the fear of right atrial obliteration by tumoral thrombus, (4) the patient’s positive test result for COVID-19, and (5) the context of the COVID-19 pandemic. Cardiac surgery was performed first. The patient underwent coronary bypass (May 2020), and thrombectomy was performed through a right atriotomy up to the level of the hepatic vein (Figure 2).
The patient’s postoperative course was complicated by acute renal failure and mild hepatic failure. Dialysis was started for 2 months and was gradually dis- continued thereafter. The patient underwent right portal vein embolization (July 2020), which was followed 8 weeks later by right hepatectomy (September 2020) that extended to segment 1 and the retrohepatic inferior vena cava under venovenous bypass. Caval replacement was achieved by a Gore-Tex (W.L. Gore & Associates) pros- thesis that was 20 cm long and 20 mm wide.
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Pathologic examination revealed adrenocortical car- cinoma with a tumoral thrombus. The patient was administered adjuvant mitotane. Consent from the pa- tient was obtained to publish this case. In November 2021, 18 months after cardiac surgery and 14 months after liver surgery, the patient showed no evidence of recurrent disease in the most recent positron emission tomographic scan.
COMMENT
Adrenocortical carcinoma can extend through the adre- nal veins directly into the caval system. Removal of the intracardiac extension is necessary to avoid atresia of the tricuspid valve, which can lead to death. The pres- ence of tumoral thrombosis represents a poor prognostic factor, but long-term survival has been reported after radical resection in such patients.3 Extension to the right atrium requires a careful surgical strategy. A thor- acoabdominal approach with cardiopulmonary bypass has been described in these conditions, and thrombec- tomy is performed either en bloc or in 2 pieces after adrenalectomy during the same operation.
In the case presented, a 2-stage approach was cho- sen to avoid to reduce the chance of mortality of the procedure because of the relative urgency of removing the atrial thrombus, the need for liver hypertrophy
before major liver resection, and the use of systemic anticoagulation during major liver resection. It could be argued that atrial thrombectomy followed by delayed removal of the primary tumor can impair overall survival. However, en bloc surgery was considered too risky in this case, in which urgency was necessitated by cardiac symptoms and not by removal of the primary tumor. Additionally, the pa- tient needed portal vein embolization to prepare the volume of the future liver remnant adequately before the major liver resection.
Although en bloc resection should be favored when- ever possible, the presented approach could be useful when combined surgery is considered too risky. The need for portal vein embolization to induce right liver hypertrophy (which needs 4-6 weeks to develop), as in our case, may be a good indication for this approach in patients with relative urgency for cardiac surgery. The risk of increased bleeding under anticoagulation could further increase the difficulty of surgery when major liver resection is also indicated. Nevertheless, this approach will need further experience and longer follow-up to be validated.
In conclusion, removal of the intracardiac extension of abdominal malignant disease can be performed in a 2- stage procedure to reduce the risk of simultaneous sur- gery in challenging situations.
REFERENCES
1. Chiappini B, Savini C, Marinelli G, et al. Cavoatrial tumor thrombus: single-stage surgical approach with profound hypothermia and circulatory arrest, including a review of the literature. J Thorac Cardiovasc Surg. 2002;124:684-688.
2. Tsuji Y, Goto A, Hara I, et al. Renal cell carcinoma with extension of tumor thrombus into the vena cava: surgical strategy and prognosis. J Vasc Surg. 2001;33:789-796.
3. Lau C, O’Malley P, Gaudino M, Scherr DS, Girardi LN. Resection of intraabdominal tumors with cavoatrial extension using deep hypothermic circulatory arrest. Ann Thorac Surg. 2016;102:836-842.