Ann Thorac Surg 2005;79:1040-1042
© 2005 The Society of Thoracic Surgeons
Case report
Cardiac Lymphoma Following Mitral Valve Replacement
Nicolas M. Dürrleman, MDa,
Ismaïl El-Hamamsy, MDa,
Roland G. Demaria, MD, PhDa,
Michel Carrier, MDa,
Louis P. Perrault, MD, PhDa,*,
Bernard Albat, MDa
a Department of Cardiovascular Surgery, Montreal Heart Institute, Montreal, Quebec, Canada, and Department of Cardiovascular and Thoracic Surgery, CHU Arnaud de Villeneuve, Montpellier, France
Accepted for publication September 10, 2003.
* Address reprint requests to Dr Perrault, Department of Surgery, Montreal Heart Institute, 5000 Belanger St E, Montreal, Quebec H1T 1C8, Canada
lpperrau{at}icm.umontreal.ca
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Abstract
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A primary cardiac lymphoma was encountered in a patient with prosthetic mitral valve dysfunction. Although rare, this tumor could be related to the presence of prosthetic material and specifically that of Dacron. Primary cardiac tumors have to be considered in the differential diagnosis of prosthetic valve dysfunction.
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Introduction
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Primary malignant cardiac tumors are extremely rare. A primary cardiac lymphoma nine years following mitral prosthetic valve implantation was encountered. The tumor developed in contact with the Dacron prosthetic ring. A few cases of sarcomas originating in close contact with this polymer have been reported. We therefore discuss the potential carcinogenic role of Dacron in the pathogenesis of malignant tumors.
A 65-year-old patient with a mechanical mitral valve prosthesis and a well-tolerated refractory anemia, presented with a 6-week history of difficulty adjust-ing her anticoagulant therapy. She had no prior medical history other than colic melanosis with lymphangiectasias.
The patient was hospitalized in the coronary care unit (CCU) following an episode of left heart failure. Clinical examination was normal and no new heart sounds or murmurs were heard. Electrocardiogram revealed a left bundle branch block. Bilateral signs of pulmonary edema were seen on the chest roentgenogram. Transthoracic echocardiography revealed abnormally elevated mitral transprosthetic gradient at 20 mm Hg with severe left ventricular failure (30% of ejection fraction). Transesophageal echocardiography (TEE) confirmed the presence of a voluminous, mobile obstructive thrombus on the atrial side of the mitral prosthesis. A suspicion of acute prosthetic thrombosis was therefore raised and surgery was undertaken. Through a median sternotomy and using hypothermic bypass and crystalloid cardioplegia, a left atriotomy was performed. A 3.5-cm heterogenous and pedicled tumor was seen originating from the Dacron ring and the atrial endocardium. The tumor, believed to be a cardiac myxoma, was resected along with its pedicle. No pannus or atrial or valvular thrombi were observed. The mitral prosthesis was left in place after a thorough cleaning of the cavity and examination of valve function. The cross-clamp time was 40 minutes and cardiopulmonary bypass time was 103 minutes.
Pathologic examination (Fig 1) revealed a 4 x 2 x 2.5 cm giant cell centroblastic malignant lymphoma, encapsulated in an old organized thrombus. Immunohistochemistry confirmed the lymphomatous nature of the tumor with positive leukocytosis antigen. Its implantation base was at the endocardial level. The postoperative period was uneventful. An extensive investigation, including bone nuclear scan and magnetic resonance imaging, revealed no other sites of lymphomatous disease. Control TEE showed a normally functioning prosthetic valve. After discussion with the patient, the latter declined adjuvant chemotherapy or radiotherapy. Ten months later, the patient was rehospitalized for weakness, weight loss, and spiking fever. A palpable periumbilical mass was found on physical examination, which was confirmed by abdominal scan showing a large retropancreatic mass (9 x 15 x 8 cm), and numerous periaortic adenopathies. The final diagnosis was a secondary digestive lymphoma. The TEE remained negative at that time. Chemotherapy was initiated but the patient developed multiple complications and died two months later.
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Comment
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Primary malignant cardiac tumors are a rare occurrence varying from 0.002% to 0.3% in autopsy series [1], partially explained by the low mitotic activity of cardiac cells [2]. They represent 25% of all encountered primary cardiac tumors [3], the rest being benign lesions (mostly cardiac myxomas). Cardiac lymphomas represent 1.5% to 5% of all primary malignant cardiac tumors. They were first described in 1885 and are thought to originate from the cardiac lymphoid tissue. Immunosuppressed patients are most vulnerable to developing such tumors [4].
Determining whether the cardiac lymphoma is a primary tumor or a metastatic lesion from a systemic primary site is always challenging. Several features favor the metastatic nature of a cardiac tumor including a preferential epicardial localization and frequent pericardial involvement. Cardiac lymphomas are most commonly located in the right atrium and very often clinically silent until a very advanced stage of the disease. The absence of all these features in the present case, the negative full body workup at the time of presentation, and the latency before the abdominal expression of the disease strongly suggest the primary nature of the observed cardiac tumor.
The occurrence of a primary cardiac lymphoma following prosthetic valve replacement could be related to the potential carcinogenic role of the Dacron annulus on the prosthetic rings. Indeed, many of the polymeric materials used in surgery have been shown to induce tumor development in animals [5, 6]. However, only a handful of cases have been reported in humans, mostly related to Dacron vascular grafts or cardiac prosthesis [7, 8]. Oppenheimer and colleagues [5, 6] demonstrated the induction of sarcomas following subcutaneous implantation of polymers with a latency period of 1 to 2 years. Furthermore, the three-dimensional structure of the polymer is an important contributing factor. Therefore, a link was suspected in the present case due to the presence of Dacron material and the lymphoma developing in close contact to it. To our knowledge, this case is the second one to be reported in the world literature [9].
In conclusion, although primary cardiac lymphomas are a rare occurrence, they have to be considered along with thrombosis in the differential diagnosis of patients presenting with acute prosthetic valve dysfunction.
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References
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Abstract
Introduction
