Interactive Cardiovascular and Thoracic Surgery 2:38-39(2003)
© 2003 European Association of Cardio-Thoracic Surgery
Case report - Cardiac general |
Right atrial hemangioma: clinicopathological considerations of a case
Augusto Orlandia,*,
Amedeo Ferlosioa,
Antonio Pellegrinob and
Luigi Giusto Spagnolia
a Department of Biopathology, Anatomic Pathology Institute, Tor Vergata University, Via della Ricerca Scientifica 00133, Rome, Italy
b Cardiovascular Surgery Institute, Tor Vergata University, Rome, Italy
* Corresponding author. Tel.: +39-6-72594232; fax: +39-6-72594230
orlandi{at}uniroma2.it
Received July 11, 2002;
received in revised form October 25, 2002;
accepted November 4, 2002
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Abstract
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Hemangiomas are rare benign tumours of the heart, generally asymptomatic and usually diagnosed in male young or full-grown adults. We report here a case of a symptomatic right atrial hemangioma in a 75-year-old woman with a clinical history of hypertension. The tumour was diagnosed after an episode of acute bronchopneumonia precipiting the patient's cardiac failure initially referred to the hypertensive condition.
Key Words: Cardiac hemangioma; Right atrium; Heart failure
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1. Introduction
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With an incidence varying from 0.0017 to 0.28% in selected autopsy series [1,2], primary tumours of the heart are very uncommon. Hemangioma is a rare cardiac tumour predominantly composed of blood vessels, with an incidence varying from 5 to 10% in surgically reported series [3,4]. Cardiac hemangiomas occur in patients of all ages with a male predominance and a mean age at clinical presentation of 43 years [3]. We describe here a right atrial hemangioma in a 75-year-old woman whose symptoms were initially referred to the patient's cardiac failure.
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2. Case report
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A 75-year-old woman with a clinical history of hypertension was admitted for an acute episode of dyspnoea and cough. She also presented fever, general illness, joints and thoracic pain. X-rays showed a marked pleural effusion of the left pulmonary field and evidence of bilateral bronchopneumonia. The heart had no relevant alteration in shape and dimensions. An electrocardiogram showed non-specific alterations of T-wave and S-T tract. On the evidence of acute pulmonary infective disease, a pharmacological therapy was decided. The treatment successfully eliminated the feverish symptoms, but the patient still presented cough, exertional dyspnoea and, in addition, hepatomegaly and declivous oedema. On the evidence of precipiting cardiac failure, a hemodynamic study was performed. A two-dimensional echocardiography (Fig. 1) revealed a pronounced pericardial effusion and a right atrial mass in a non-homogeneous pattern, apparently capsulated, ovoid in shape, originating from the inter-atrium septum and largely involving the roof of the cardiac chamber. The tumour obstructed right atrial filling from cavae. A cardiac catheterization showed high visualization of the tumour. The patient was classified as NYHA-2 and Canadian-1 and a surgical treatment decided. The surgical access was performed through a medial sternotomy and a standard lateral right atriotomy was made. The tumour was completely removed and atrium rebuilt with an autologal pericardial patch. The surgical treatment required a cardiopulmonary by-pass and low–moderate hypothermia. Macroscopically, the tumour was 4x5x3.5cm in diameter, grey, ovoid in shape and well capsulated. The cut surface was bleeding. Histologic examination showed a typical cavernous-capillary hemangioma (Fig. 2a). The tumour was composed of open capillary channels, dilated cavernous vessels and lobules of endothelial cells with scattered pericytes and fibroblasts. Immunohistochemical results showed diffuse staining for CD34 lining vascular spaces (Fig. 2b).
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Fig. 1 Two-dimensional sub-costal echocardiography showing the right atrium (RA) occupied by tumoural mass (arrow), the pericardial effusion and hepatic congestion (LA, left atrium; LV, left ventriculum; RV, right ventriculum).
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Fig. 2 (a) Microscopic examination showing the typical cavernous-capillary aspect of the cardiac hemangioma (original magnification 100x); (b) CD34 immunostaining demonstrates the endothelial origin of cells covering fibrovascular spaces of the tumour (original magnification 250x).
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3. Comments
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Hemangioma is a very uncommon primary tumour of the heart, with only less than 40 cases being described in literature. It originates from vascular cells and the histologic appearance varies from a prevalent capillary to a cavernous aspect [3]. Anatomically, all cardiac chambers should be involved, but is more frequent in the right atrium [4]. Hemangioma is usually asymptomatic even though it sometimes produces symptoms such as arrhythmias, pericardial effusion, sudden death, congestive heart failure and dyspnoea on exertion [3]. These symptoms are generally observed in young or full-grown adults and largely depend on the localization and/or the cardiac structures involved [3,4]. The diagnosis is performed by echocardiography and confirmed by histologic examination. Hemangiomas usually arise from the posterosuperior part of right atrium, close to superior vena cava and compressing it without sign of invasion [5]. Differential echocardiographic diagnosis is usually made with cardiac myxoma that arises from fossa ovalis of inter-atrium septum. Unlike hemangioma, myxoma is sided in most of the cases in the left atrium, pedunculated and mobile, without any sign of atrial wall invasion [5]. The clinical relevance of this case of symptomatic hemangioma depends on the advanced age at the clinical presentation of the tumour that prevails among male young and middle-aged patients, and the female sex [3]. Only few cases of symptomatic cardiac hemangioma are described in late elderly patients and all men [6,7], since age-related cardiac failure may hide the presence of the tumour.
doi:10.1016/S1569-9293(02)00095-6
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References
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Abstract
1. Introduction