Ann Thorac Surg 2010;90:642-644. doi:10.1016/j.athoracsur.2010.02.023
© 2010 The Society of Thoracic Surgeons
Case Reports
Vena Cava Sarcoma With Tumor Embolus in the Pulmonary Artery: Surgical Treatment
Tobias Krüger, MDa,*,
Migdat Mustafi, MDa,
Silvio Nadalin, MDb,
Hermann Aebert, MDa
a Department of Thoracic, Cardiac and Vascular Surgery, University Hospital of Tübingen, Tübingen, Germany
b Department of General, Visceral and Transplant Surgery, University Hospital of Tübingen, Tübingen, Germany
Accepted for publication February 5, 2010.
* Address correspondence to Dr Krüger, Department of Thoracic, Cardiac and Vascular Surgery, University of Tübingen, Hoppe-Seyler-Str. 3, 72076 Tübingen, Germany (Email: krueger-tobias{at}web.de).
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Abstract
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Tumors of the inferior vena cava are rarely complicated by pulmonary tumor embolism. We report a patient with a leiomyosarcoma of the inferior vena cava complicated by an embolus in the left pulmonary artery. We describe the successful treatment of this condition, which consisted of cavoplasty and pulmonary endarterectomy conducted under hypothermic circulatory arrest. Different aspects of the surgical strategy are discussed.
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Introduction
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Leiomyosarcoma of the inferior vena cava (IVC) is a rare tumor; however, it is the most frequent primary neoplasm of this vessel [1]. Pulmonary tumor embolism in IVC tumors is a rare occurrence. The comorbidity of an IVC tumor and an embolus in the pulmonary vasculature represents a therapeutic challenge.
We present the case of a 49-year-old man with a leiomyosarcoma of the IVC and a tumor embolus in the left pulmonary artery (PA) treated by radical resection under hypothermic circulatory arrest (HCA). Two years before admission, the patient was spuriously diagnosed with pulmonary embolism and a combined thrombophilic diathesis. A tumor of the IVC was detected at the follow-up 1 year later. Analysis of the biopsy specimen revealed a leiomyosarcoma grade 2.
At the time of the operation, the tumor in the IVC spanned from the confluence of the right renal vein to the inflow of the right atrium (RA), causing subtotal occlusion of the IVC (Fig 1A). Because of constant growth, the structure in the pulmonary vasculature was likely to be a tumor as well. The left PA was completely obstructed by the tumor embolus, which extended into the lobar branches of the PA, mainly into the inferior lobe (Fig 1B). The measured PA pressure was normal. The patient reported minor dyspnea on exercise, weight loss of 4 kg in 6 months, and occasional night sweats. Otherwise, he was in good clinical condition.
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Fig 1. (A) Frontal reconstruction computed tomography image showing the inferior vena cava tumor. (B) Transversal computed tomography angiography shows a tumor embolus in the left pulmonary artery.
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Surgical exposure was accomplished through a sternotomy and median laparotomy. The IVC was exposed by complete mobilization of the right hemiliver, transecting the falciform and the coronary ligaments. The renal veins and the infrarenal IVC were controlled by vessel loops, and the left PA was dissected to its lobar branches.
After cannulation of the aorta and the right auricle (single-stage), cardiopulmonary bypass (CPB) was established. Cardioplegia was administered, and HCA was initiated at a tympanion temperature of 20°C.
The RA and the IVC were opened through a lateral longitudinal incision reaching from the diaphragm to the renal veins (Fig 2A). The tumor infiltrated the dorsal portion of the suprarenal IVC with a length of 3 cm; a smaller area of infiltration was located at the level of the diaphragm. The infiltrated vascular wall was excised completely. Subsequently, the IVC was reconstructed using a native pericardial patch sutured with 4-0 polypropylene (Fig 2B). The IVC intervention was done during two phases of HCA of 25 minutes and 16 minutes, interrupted by 5 minutes of perfusion.
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Fig 2. Intraoperative views show (A) the inferior vena cava with luminal tumor, (B) the reconstructed inferior vena cava, (C) the left pulmonary artery with tumor embolus, and (D) the resected inferior vena cava tumor.
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After the IVC reconstruction, CPB was continued for 10 minutes. Then, the pulmonary trunk was clamped and the left PA was incised longitudinally (Fig 2C). During another 23 minutes of HCA, a tumor thromboendarterectomy was done. The central parts of the tumor could be removed easily; whereas, removal in the inferior lobe was possible only by circular dissection of the intima far into the segmental arteries. All visible tumor was resected completely. The PA was directly closed with a 5-0 polypropylene suture.
After reperfusion and rewarming, the patient was weaned from CPB without problems. At the end of the operation, the patient was hemodynamically stable without catecholamines. The patient received 500 mL of packed red blood cells and 600 mL of plasma.
The patient was extubated at 12 hours postoperatively. His postoperative course was complicated by bronchopneumonia and a transitory psychotic syndrome. The patient was discharged on postoperative day 20.
The histopathologic workup showed that the surgical margins were negative in the IVC (R0). In the pulmonary endarterectomy specimen, the neoplastic nature of the embolus was proven; however, margins could not be judged properly.
On follow-up visitation at 6 months, the patient was in a good clinical condition, without any complaints, and had returned to work. The follow-up computed tomography imaging showed no tumor recurrence or metastasis. The reconstructed IVC was patent.
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Comment
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The IVC is subdivided into 3 segments delineated by the inflow of the hepatic and renal veins [2]. Tumors of the inferior or middle segment can be resected during clamping of the IVC and by performing the Pringle maneuver. As soon as the upper segment or the RA is involved, CPB is mandatory.
The techniques used for the resection and reconstruction of the IVC include ligation, graft replacement [3], patch cavoplasty, or primary repair [2]; however, the choice of techniques to be used is controversial. If perivascular infiltration is observed, a preferably radical approach with wide resection and ligation or replacement of the IVC is mandatory [4]. However, a predominantly intravascular growth pattern is shown in a relevant portion of leiomyosarcomas [2]. In this patient, no perivascular infiltration was seen intraoperatively or during the pathologic workup; therefore, we determined that partial resection with patch reconstruction was adequate.
To prevent thrombosis of the reconstructed IVC, the construction of a temporary arteriovenous fistula is often advised. We and others [3] think that this is not necessary in case of suprarenal cavoplasty. In our experience of more than 30 cases of cavoplasty using a native pericardial patch, we have never seen a thrombosis; in addition, the lowest rates of leg edema are reported for patch reconstruction [2]. However, we advise anticoagulation with phenprocoumon for 3 months after IVC reconstruction.
In the described case, IVC resection and repair were performed during HCA because the suprahepatic segment and the RA had to be inspected. After RA closure, we initially tried to complete the cavoplasty during CPB; however, massive backflow through the hepatic veins and collaterals was extremely hindering and led us to complete the reconstruction during HCA. For management of possible complications, especially intraoperative tumor embolization, cannulation was done before luxation of the liver. Cerebral oxygen saturation monitoring was used to prevent critical desaturation during HCA.
Although pulmonary embolectomy for acute pulmonary embolism can be done as a beating-heart procedure, pulmonary endarterectomy is required for chronic pulmonary embolic disease, regardless of whether it is thromboembolic or neoplastic. The latter procedure usually requires HCA to facilitate a blood-free field in patients with compensatory increased blood flow in bronchial arteries and collaterals [5].
In the tumor in the pulmonary vasculature, unlike the tumor in the IVC, it was impossible to prove R0 resection by means of histopathology, whereas intraoperatively, all macroscopic parts of the tumor were removed completely. However, the patient's history and symptoms indicated a slow and less-invasive growth pattern of the tumor. Therefore, pulmonary recurrence of the tumor should be detected early by follow-up computed tomography and optionally could be treated by appropriate lung resection.
We conclude that the combined removal of a large leiomyosarcoma of the IVC and pulmonary endarterectomy for extensive intravascular neoplastic disease using the described approach of intermittent HCA is feasible and can produce a good perioperative and midterm postoperative result.
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References
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- Mingoli A, Cavallaro A, Sapienza P, Di Marzo L, Feldhaus RJ, Cavallari N. International registry of inferior vena cava leiomyosarcoma: analysis of a world series on 218 patients Anticancer Res 1996;16(5B):3201-3205.[Medline]
- Hollenbeck ST, Grobmyer SR, Kent KC, Brennan MF. Surgical treatment and outcomes of patients with primary inferior vena cava leiomyosarcoma J Am Coll Surg 2003;197:575-579.[Medline]
- Bower TC, Nagorney DM, Cherry Jr. KJ, Toomey BJ, Hallett JW, Panneton JM, et al. Replacement of the inferior vena cava for malignancy: an update J Vasc Surg 2000;31:270-281.[Medline]
- Kieffer E, Alaoui M, Piette JC, Cacoub P, Chiche L. Leiomyosarcoma of the inferior vena cava: experience in 22 cases Ann Surg 2006;244:289-295.[Medline]
- Jamieson SW, Kapelanski DP, Sakakibara N, et al. Pulmonary endarterectomy: experience and lessons learned in 1,500 cases Ann Thorac Surg 2003;76:1457-1462.[Abstract/Free Full Text]
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Abstract
Introduction
