Ann Thorac Surg 2005;80:e8-e9
© 2005 The Society of Thoracic Surgeons
Case report
Left Atrial Extension of a Wilms' Tumor
Gonçalo F. Coutinho, MD,
Fátima Heitor, MD,
Pedro E. Antunes, MD*,
Manuel J. Antunes, MD, PhD
Department of Cardiothoracic Surgery, University Hospital, Coimbra, Portugal
Accepted for publication April 22, 2005.
* Address reprint requests to Dr Antunes, Cirurgia Cardiotorácica, Hospitais da Universidade, Coimbra 3000-275 Portugal (Email: antunes.cct.huc{at}sapo.pt).
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Abstract
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Isolated involvement of the left heart chambers by metastases of malignant tumors is extremely rare. We report a case of an 8-year-old child, with a left atrial metastasis of a Wilms' tumor detected in a control nuclear magnetic resonance 1 year after left inferior lobectomy for metastatic extension of the kidney tumor, which was diagnosed 4 years earlier. The metastasis was excised en bloc with a disc of the posterior left atrial wall.
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Introduction
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Wilms' tumors most commonly occur between the ages of 2 and 5 years and are the second most common abdominal tumor in children, secondary to neuroblastomas [1]. Although primary renal carcinomas commonly extend into the inferior vena cava and occasionally reach the right atrium, our literature search found only a few reported instances of surgical resection of metastatic tumors extending into the left atrium through the pulmonary veins and none concerning a Wilms' tumor [2\#8211;5]. We report a case of metastatic involvement of the left atrium occurring 4 years after nephrectomy and 1 year after left inferior lobectomy for an initial metastasis.
A 4-year-old female was diagnosed with a stage II Wilms' tumor of the left kidney in 2001 and had a nephrectomy, after which she was submitted to the treatment regimen proposed by the National Wilms Tumor Study 5-C. She remained free from identifiable disease for more than 1 year, when she was then diagnosed as having a relapse of the disease in the left pulmonary hilum with extension to the mediastinum and compression of the left atrium. She repeated the therapeutic protocol with good response and a left inferior lobectomy was performed at another hospital in January 2003. She continued with radiotherapy and chemotherapy for nearly 3 months, which was stopped after a positron-emission tomographic examination showed no evidence of residual disease.
Serial controls were negative until September 2004, when a lesion in the left atrial wall was observed in a computed tomographic scan of the chest. The National Wilms Tumor Study 5-C confirmed the existence of a left atrial mass, suspected of infiltration of the left atrial wall and the descending aorta (Fig 1). Transesophageal echocardiography revealed a pedunculated, irregular mass, suggestive of involvement of the posterior left atrial wall, but apparently without extension to the pulmonary veins. The mass bounced into the mitral valve, causing no restriction or insufficiency, but seemed highly unstable with an eminent risk of embolism. Otherwise, she was an active child, completely asymptomatic, with no other relevant physiopathologic findings.
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Fig 1. CT scan showing a posterior left atrial mass with possible infiltration of the left atrial wall and descending aorta.
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During surgery, a median sternotomy was performed, and the patient was placed on cardiopulmonary bypass and cooled down to 28°C, and cold cardioplegia was administered by anterograde. A left atriotomy was made parallel and posterior to the interatrial sulcus. A pedunculated mass (3 to 4 cm in diameter) was identified, which was located between the superior pulmonary veins but without involving them (Fig 2). At sight and touch, the posterior atrial wall did not appear infiltrated. Despite the friable appearance, the tumor was consistent and did not fragment when pulled. It was excised with a margin of the posterior left atrial wall, and the defect created could be closed, tension-free, with a running suture of 4-0 polypropylene.
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Fig 2. Intraoperative photograph showing a pedunculated mass (3 to 4 cm in diameter) located between the superior pulmonary veins.
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Histologic examination revealed a myocardial metastasis of Wilms' tumor with a blastomatous pattern. The fragment of pericardium and atrial wall marginal to the mass showed no disease. Post-excision transesophageal echocardiography showed no residual lesion. The patient had an uneventful recovery and remains well and free from the tumor.
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Comment
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Wilms' tumor is curable in the majority of affected children. More than 90% of patients survive 4 years after the diagnosis [1]. The prognosis is related not only to the stage of the disease at the time of diagnosis, the histopathologic features, patient age, and tumor size, but also to the team approach to each patient by the pediatric surgeon, radiation oncologist, and pediatric oncologist [6\#8211;7].
Metastatic tumors of the heart are 20 to 40 times more common than primary tumors. Melanoma, leukemia, lymphoma, and lung and breast cancers metastasize to the heart with the highest frequency. These metastases involve the pericardium, myocardium, and endocardium in decreasing order of frequency [4]. Metastatic tumors may reach the heart by hematogenous or lymphatic spread, or by direct invasion. Intracavitary tumors may also arise from tumor thrombus growing through the lumen of the great veins. Tumor growth in the lumen of the vein may induce deposition of fibrin, which then serves as a framework to support cancer growth [8].
The case we report herein is probably related to the latter (ie, implantation of tumor thrombus), because there was a previous metastasis of the lung and the pulmonary veins were free from disease at the time of surgery. Commonly, extension to the heart of Wilms' tumors occurs through the lumen of the inferior vena cava. Involvement of the heart in the absence of direct caval extension is extremely rare. Isolated left heart involvement is exceedingly rare, and that is what makes this case unique.
Despite the dismal prognosis of secondary tumors, the expected survival of stage IV (free from disease at 4 years in this case) is 73% according to the National Wilms Tumor Study 5-C report. Because the current patient was successfully and apparently treated by a complete surgical resection, it may be reasonably expected that she has been cured.
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References
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- Hay Jr WW, Hayward AR, Levin MJ, Sandheimer JM. Current paediatric diagnosis and treatment. 14th ed.. Norwalk, CT: Appleton & Lange; 1999. pp. 787-789.
- Kodoma K, Doi O, Tatsuta M. Unusual extension of lung cancer into the left atrium via the pulmonary veins Int Surg 1990;75:22-26.[Medline]
- Bradley SM, Bolling SF. Late renal cell carcinoma metastasis to the left ventricular outflow tract Ann Thorac Surg 1995;60:204-206.[Abstract/Free Full Text]
- Patane J, Flum DR, Mcginn Jr JT, Tyras DH. Surgical approach for renal cell carcinoma metastatic to the left atrium Ann Thorac Surg 1996;62:891-892.[Abstract/Free Full Text]
- Schuman R. Primary pulmonary sarcoma with left atrial extension via the left superior veins J Cardiovasc Surg 1984;88:189-192.
- Ritchey ML, Haase GM, Shochat S. Current management of Wilms' tumor Semin Surg Oncol 1993;9:502-509.[Medline]
- Beslow N, Sharples K, Beckwith JB, et al. Prognostic factors in nonmetastatic, favourable histology Wilms' tumour. Results of the Third National Wilms' Tumor Study Cancer 1991;68:2345-2353.[Medline]
- Reardon MJ, Smythe WR. Cardiac NeoplasmsIn: Cohn LH, Edmunds Jr LH, editors. Cardiac surgery in the adult, 2nd ed. New York: McGraw Hill; 2003. pp. 1373-1400.
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Abstract
Introduction
