Eur J Cardiothorac Surg 2008;34:697-699. doi:10.1016/j.ejcts.2008.05.040
Copyright © 2008, European Association for Cardio-thoracic Surgery. Published by Elsevier. All rights reserved.
Intracardiac renal cell carcinoma metastasis
Frédéric Cochenneca,c,*,
Agathe Seguina,c,
Marc Riquetb,c,
Jean-Noël Fabiania,c
a AP-HP, Hôpital Européen Georges Pompidou, Service de Chirurgie Cardio-Vasculaire, France
b AP-HP, Hôpital Européen Georges Pompidou, Service de Chirurgie Thoracique, France
c Université Paris V, Faculté de Médecine, 20 rue Leblanc, 75015 Paris, France
Received 28 December 2007;
received in revised form 13 May 2008;
accepted 21 May 2008.
* Corresponding author. Address: Hôpital Européen Georges Pompidou, Service de Chirurgie Cardio-Vasculaire, 20 rue Leblanc, 75015 Paris, France. Tel.: +33 6 08046385; fax: +33 1 48955230. (Email: frederic.cochennec{at}wanadoo.fr).
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Abstract
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Cardiac involvement by intravascular protruding renal cell carcinoma is a well-recognised phenomenon. It most commonly occurs through inferior vena cava extension. Here, we report a case of a lower lobar lung metastasis from renal cell carcinoma involving the left atrium via the inferior pulmonary vein in a patient presenting with von Hippel–Lindau disease. Complete surgical resection was achieved under cardiopulmonary bypass.
Key Words: Lung • Cancer • Metastasis • Cardiac intervention • Surgery
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1. Case report
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von Hippel–Lindau disease (VHL) is a dominantly inherited familial cancer syndrome caused by germline mutations in the VHL tumour suppressor gene [1]. Renal cell carcinoma occurrence is frequent in this disease. We report a case of bilateral renal cell carcinoma (RCC) with left atrial metastatic involvement.
A 42-year-old woman was referred to our institution in March 2006 for evaluation of a mass located within the left atrium (LA). Her past medical history was notable for multiple retinal and cerebral haemangioblastomas related to a VHL. She also underwent left and right nephrectomy in 2002 and 2004 respectively for RCC and since 2004 was on haemodialysis. Her family was known to be affected by VHL, with five affected members.
On presentation, she was asymptomatic. Physical examination was non-contributory. Electrocardiogram and laboratory findings were normal. Chest X-ray showed a large mass in the left lower lobe. A chest and abdominal computed tomography (CT) scan revealed a 50 mm x 60 mm tumour in the left lower lobe extending intraluminally from the left inferior pulmonary vein into the LA (Fig. 1
). Two nodules in the right upper lobe, and two soft tissue lesions of the liver consistent with metastases were also discovered.
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Fig. 1. (A) Axial contrast-enhanced computed tomography shows a mass involving the left lower lobe and extending into the left atrium through the left inferior pulmonary vein. (B) FDG-PET scan reveals a hypermetabolic soft tissue mass of the left lower lobe and a tubular extension of increased radiotracer uptake in the LA. Right upper lobe and liver metastases are also visualised.
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A transoesophageal echocardiogram revealed a multilobed mass of homogeneous echogenicity, undulating from the left inferior pulmonary vein to the mitral valve orifice without involving it. Myocardial function was normal. Subsequent F-18 FDG PET confirmed the hypermetabolic pattern of the lung, left atrial and liver lesions. Bronchoscopy showed a hypervascular endobronchial lesion arising from the left lower bronchial tree. Biopsy was not obtained due to risk of haemorrhage. A percutaneous CT-scan guided biopsy of the left lower lobe mass confirmed the diagnosis of metastatic RCC. Despite of the metastatic status of the patient, surgical resection was scheduled in regard of systemic embolic risk and acute mitral obstruction.
A median sternotomy combined with a left anterolateral thoracotomy was performed. Segmental arterial branches and bronchus going to the left lower lobe were dissected and divided so that the left lower lobe was pedicled on the left inferior pulmonary vein. Cardiopulmonary bypass was then established by cannulating the ascending aorta and the superior and inferior vena cava. A normothermic blood cardioplegia was performed. A left atriotomy revealed a bulging mass extending into the lumen of the left inferior pulmonary vein. En bloc resection (left lower lobectomy associated with the protruding tumoural mass resection) was achieved without difficulty. The left atriotomy was closed and cardiopulmonary bypass stopped. Histologic examination confirmed the diagnosis of metastasis from a RCC. The tumour tissue demonstrated positive staining for cytokeratin AE1/AE3, vimentin and CD10 and negative staining for cytokeratin 7 and cytokeratin 20. The patient was extubated 12 h after the operation and postoperative recovery was uneventful. Six months after the operation she was doing well without local recurrence on follow-up chest CT-scan and under systemic interferon treatment. Eight months later she unfortunately developed a brain metastasis treated by cerebral irradiation and died in November 2006.
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2. Comment
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Cardiac involvement from RCC is a well-recognised entity with several cases reported in recent literature. In the great majority of cases, the tumour reaches the right cavities through a neoplastic thrombus within the inferior vena cava (IVC) [2]. Cardiac metastasis from RCC without vena cava involvement is extremely rare. However, this can occur through systemic haematogenous spreading or by direct extension from either mediastinal or lung lesions involving a pulmonary vein. Although extension of primary pulmonary malignancies into the LA by invasion of the pulmonary veins is well documented, this event is uncommon regarding lung or mediastinal metastatic lesions. Concerning RCC, only three previous cases have been reported [3–5] (Table 1
).
Such lesions may result in systemic embolisation, syncope [3,4] owing to the prolapse of the tumour into the mitral orifice, arrhythmia, or congestive heart failure due to obstruction of the pulmonary venous outflow. However, they often remain silent making their early recognition and diagnosis extremely difficult preoperatively. In our case, transoesophageal echocardiography and CT scan both identified the left atrial mass, but did not allow for clear differentiation between bland thrombus and tumour tissue. The PET scan demonstrated a left thoracic area of markedly increased FDG uptake, corresponding to the left lower lobe tumour (Fig. 1). In addition, a tubular extension of a markedly increased radiotracer uptake was continuous toward the right hilum and into an area corresponding to the LA. As documented in other reports [6], this finding is a non-specific but strong indicator of tumour tissue. In a meta-analysis evaluating 18F-FDG PET in patients with distant metastasis from RCC, the global values of sensitivity and specificity were 72% and 70 % respectively [7]. These results suggest that the performance of 18F-FDG PET in the evaluation of metastatic RCC is still limited.
This case presented an additional interest by the association with VHL. Apart from RCC, the most common tumours are haemangioblastomas of the retina and central nervous system, renal cysts, phaeochromocytomas and paragangliomas, neuroendocrine pancreatic tumours and pancreatic cysts [1]. To our knowledge, this is the first report of a lung RCC metastasis involving the LA via a pulmonary vein in a patient diagnosed to have VHL. Compared with its sporadic counterpart, some authors have advocated that RCC in VHL was associated with slower growth rates, rare metastasis, and improved cancer-specific survival [8]. This less aggressive course was not observed in this patient.
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Acknowledgments
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We thank Elisabeth Fabre-Guillevin, Patrick Bruneval, Thomas Matthiesen and Ali Al Sheri for their helpful advice and critical review of this manuscript.
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References
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- Staehler G, Brkovic D. The role of radical surgery for renal cell carcinoma with extension into the vena cava. J Urol 2000;163:1671-1675.[CrossRef][Medline]
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- Miyamoto MI, Picard MH. Left atrial mass caused by metastatic renal cell carcinoma: an unusual site of tumor involvement mimicking myxoma. J Am Soc Echocardiogr 2002;15:847-848.[CrossRef][Medline]
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- Neumann HP, Bender BU, Berger DP, Laubenberger J, Schultze-Seemann W, Wetterauer U, Ferstl FJ, Herbst EW, Schwarzkopf G, Hes FJ, Lips CJ, Lamiell JM, Masek O, Riegler P, Mueller B, Glavac D, Brauch H. Prevalence, morphology and biology of renal cell carcinoma in von Hippel–Lindau disease compared to sporadic renal cell carcinoma. J Urol 1998;160:1248-1254.[CrossRef][Medline]
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Abstract
1. Case report
