Ann Thorac Surg 2003;76:1721-1722
© 2003 The Society of Thoracic Surgeons
Case report
Metastatic carcinoid tumor of the heart
Jon O. Wee, MDa,
Jerome D. Sepic, MDa,
Tomislav Mihaljevic, MD*a,
Lawrence H. Cohn, MDa
a Division of Cardiac Surgery, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts, USA
Accepted for publication March 28, 2003.
* Address reprint requests to Dr Mihaljevic, Division of Cardiac Surgery, Brigham and Women’s Hospital, 75 Francis St, Boston, MA, USA 02115
e-mail: tmihaljevic{at}partners.org
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Abstract
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Although carcinoid heart disease has been well described in the literature, metastatic implantation in the heart is rare. We describe a 79-year-old man with no previous history of cancer who presented with progressive dyspnea. He was found to have a septal implantation of a previously undiagnosed metastatic carcinoid tumor. He underwent successful resection with an uneventful postoperative course.
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Introduction
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The heart is a rare location for neoplastic lesions. Prior to 1966, the incidence of metastases to the heart was between 0.2% and 6% in selected reports [1]. With the advent of better chemotherapy and radiation, patients have improved survival. With increased longevity, the incidence of cardiac metastases has increased to slightly above 10%. The most common malignancies include leukemia, lymphoma, and melanoma [1]. However, carcinoid manifestations of heart disease are usually due to circulating humoral mediators. Cardiac implantation of carcinoid tumors is a rare event. Here we report a case of a 79-year-old man who presented with increasing dyspnea and was found to have a septal implantation of a carcinoid tumor.
A 79-year-old man with no previous history of cancer presented with exertional dyspnea and cyanosis. The patient had a history of sick sinus syndrome, heart block, and the placement of a pacemaker. He also had at least three bowel obstructions in the past, each requiring admission to the hospital for 2 to 3 days, with each resolving spontaneously. Further investigation of the obstruction included a colonoscopy and computed tomographic scans that only demonstrated a stable 3.5 cm liver mass, but no clear cause of obstruction. In recent years, he began to experience increasing dyspnea on exertion. He did not report any flushing or diarrhea symptoms. An echocardiogram revealed severe abnormality of septal motion. A large ovoid intramural mass measuring 3 cm in diameter was noted in the right ventricular cavity adjacent to and confluent with the septal musculature (Fig 1).
The aortic valve demonstrated moderate thickening and moderate calcifications with trace insufficiency. The mitral valve was structurally normal with mild to moderate regurgitation. The left atrium was moderately dilated. The tricuspid valve showed mild to moderate regurgitation and some evidence of right ventricular hypertrophy. An endomyocardial biopsy was then performed and at that time seemed most consistent with a myxoma.
At operation, a 3 x 4 cm mass was excised through a right ventriculotomy. Immunoperoxidase staining was strongly positive for chromogranins, weakly positive for synaptophysin, and negative for leukocyte common antigen, cytokeratin 7, and cytokeratin 20. Final pathology was consistent with metastatic carcinoid tumor infiltrating myocardial tissue. A follow-up computed tomographic scan revealed multiple metastatic lesions in the liver of which the largest was 5.6 x 4.1 cm. A new 4.6 x 2.8 cm mesenteric mass was noted within the mesentery that extended to involve 12 to 15 cm of distal small bowel in the right lower quadrant. This was most consistent with the primary carcinoid tumor. Postoperatively the patient has been observed with close follow-up. Presently at 2 years after diagnosis, he is alive and well. Though he still has a persistent complaint of dyspnea with exertion, there is no evidence of additional cardiac implantation, tumor growth, or valvular disease.
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Comment
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Carcinoid tumors occur in approximately 1.5 per 100,000 people. Of these patients, approximately 20% to 30% have carcinoid syndrome develop [2]. Carcinoid syndrome is characterized by cutaneous flushing, secretory diarrhea, and bronchoconstriction resulting from the circulating hormones such as bradykinin and serotonin secreted by the tumor. Severe malignant syndromes will develop hemodynamic instability. Of those people with carcinoid syndrome, approximately 50% to 60% of patients have carcinoid heart disease develop, 20% of which present with heart failure as their primary symptom [2].
Heart failure is the result of valvular disease secondary to invasion of the valve by carcinoid plaques. These plaques consist of smooth muscle cells, myofibroblasts, and an endothelial cell layer that is adherent to the endocardium in valves. This results in contraction of the leaflets with resultant regurgitation [1, 2].
The severity of this valvular heart disease is proportional to the amount of circulating hormones secreted, namely 5-hydroxyindoleacetic acid, the breakdown product of serotonin. Because these hormones are detoxified in the liver and lungs, the valves most commonly affected are the tricuspid and pulmonic valves. Anderson and colleagues [2] reported 97% involvement of the tricuspid valve as opposed to 7% left-sided involvement, and Knott-Craig and colleagues [3] reported pulmonary stenosis (90%), tricuspid insufficiency (47%), and tricuspid stenosis (42%) as the most common lesions. These often require valve replacement with a mean duration of 10 months from diagnosis of carcinoid heart disease to surgery. Approximately one third of the patients with metastatic carcinoid disease die of right heart failure [3].
Although valvular abnormalities secondary to carcinoid tumors are well described, metastatic implantation of tumor in the heart is rarely described. The heart is involved in 10% of malignant neoplasms, most commonly by leukemia, melanoma, and lymphoma [1]. Carcinoid metastases are rare, with less than 10 patient reports in the literature. Pellikka and colleagues [4] reported the largest group of patients with carcinoid disease, and among 132 patients, 3 (4%) were noted to have myocardial metastases develop. One of these patients had a right ventricular wall implant and five additional small implants, but no evidence of valvular disease. Five year follow-up after surgical excision noted no further cardiac recurrence or carcinoid valvular disease development. In all 3 patients, the ileum was noted to be the primary source.
Hennigton and colleagues [5] reported an invading metastatic lesion without a known primary and without carcinoid syndrome. The tumor invaded a large portion of the left ventricle, it was inoperable, and hence it was treated with external beam radiation. Melitta and colleagues [6] reported a carcinoid metastatic lesion in the left ventricle treated with interferon 
2a and octreotide with symptomatic response.
Knott-Craig and colleagues [3] reported 2 patients who had implantation, with one patient’s implantation in the right atrium and the other patient’s implantation in both ventricles. Both patients had carcinoid valvular heart disease requiring valve replacement with improvement of symptoms. Drake and colleagues [7] reported three intracardiac metastases, two noted in the left atrium and one noted in the right ventricle.
Metastatic carcinoid lesions, though rare, can occur in any chamber of the heart. These lesions are not necessarily associated with carcinoid syndrome or valvular heart disease, and in some cases represent the presenting sign of metastatic carcinoid disease. Tumor implantation should be suspected in patients with carcinoid disease and heart failure. Although metastatic disease may limit longevity, lesions amenable to surgical excision or that show a response to adjuvant therapy may result in functional improvement in patients.
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References
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- Schoen F.J., Berger B.M., Guerina N.G. Cardiac effects of noncardiac neoplasms. Cardiol Clin 1984;2(4):657-670.[Medline]
- Anderson A.S., Krauss M.D., Lang M.R. Cardiovascular complications of malignant carcinoid disease. Am Heart J 1997;134:693-702.[Medline]
- Knott-Craig C.J., Schaff H.V., Mullany C.J., et al. Carcinoid disease of the heart: surgical management of ten patients. J Thorac Cardiovasc Surg 1992;104:475-481.[Abstract]
- Pellikka P.A., Tajik J., Khandheria B.K., et al. Carcinoid heart disease. Clinical and echocardographic spectrum in 74 patients. Circulation 1993;87:1188-1196.[Abstract/Free Full Text]
- Hennington M.H., Detterbeck F.C., Szwerc M.F., Fidler M.E. Invasive carcinoid tumor of the heart. J Surg Oncol 1997;66:264-266.[Medline]
- Melitta P., Kurtaran A., Vorbeck F., Oberhuber G., Raderer M. Small round cell tumors: case 2: myocardial metastases from a carcinoid tumor. J Clin Oncol 2000;8(7):1596-1597.
- Drake W., Jenkins P.J., Phillips R.R., et al. Intracardiac metastases from neuroendocrine tumors. Clin Endocrinol 1997;46:517-522.[Medline]
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Abstract
Introduction
