Ann Thorac Surg 2005;79:1402-1405
© 2005 The Society of Thoracic Surgeons
Case report
Epitheloid Hemangioendothelioma of the Heart in Infancy
Turkan Tansel, MD*,a,
Ümrah Aydogan, MDb,
Dilek Yilmazbayhan, MDc,
Bilge Bilgiç, MDc,
Misten Demiryont, MDc,
Ertan Onursal, MDa
a Department of Cardiovascular Surgery, Istanbul, Turkey
b Department of Pediatric Cardiology, Istanbul, Turkey
c Department of Pathology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey
Accepted for publication October 2, 2003.
* Address reprint requests to Dr Tansel, Istanbul University, Istanbul Faculty of Medicine, Department of Cardiovascular Surgery, Çapa, Istanbul 34390, Turkey
turkant{at}superonline.com
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Abstract
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We report a case of primary cardiac epithelioid hemangioendothelioma arising from the right atrium of a 2-month-old infant. The tumor was found incidentally during exploratory sternotomy for recurrent pericardial effusion. This case represents a very rare situation, because this is the youngest patient found in relevant literature, and because it involves extensive infiltration by the tumor without any development of intracardiac mass appearance.
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Introduction
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Primary tumors of the heart are rare, with an incidence of 0.0017% to 0.028% in reported series [1]. Hemangioma of the heart constitutes about 2.8% of all primary cardiac tumors [1], whereas cardiac occurrences of epithelioid hemangioendothelioma are exceptionally rare. We believe that only five cases of epithelioid hemangioendothelioma of the heart have been previously reported in the literature. We report a case of epithelioid hemangioendothelioma of the right atrium as the first description of this tumor occurring in infancy.
A 21-year-old primigravida who had no previous medical history and an uncomplicated pregnancy had presented for a routine ultrasound examination. The ultrasound examination at 34 weeks of gestation revealed the presence of a fetal pericardial effusion. A follow up was planned. At 38 weeks of gestation, a male infant weighing 3.050 kg was delivered after spontaneous rupture of the membrane. The infant showed signs of cardiorespiratory distress immediately after delivery with apnea, cyanosis, and signs of poor peripheral circulation, with a heart rate of 90 beats per minute and blood pressure of 45/30 mm Hg. No significant improvement occurred after endotracheal intubation, mechanical ventilation, and inotropic support. No murmurs were heard on auscultation. The liver was palpated 2 cm below the right costal margin. The chest roentgenogram revealed a marked enlargement of the cardiac shadow. Echocardiography demonstrated a large pericardial effusion. A pericardiocentesis was performed and 150 mL of serous fluid was withdrawn, which showed no growth of viruses, bacteria, or malignant cells. The infant's hemodynamic and respiratory condition improved immediately after the procedure. Pericardiocentesis was repeated on the following day and at the third week of life due to recurring signs and symptoms of cardiac tamponade. A follow-up transthoracic echocardiography done at the fourth week demonstrated no intrapericardial or intracardiac mass, as well as no intracardiac defect. Multiple procedures of pericardiocentesis had to be performed during the next 4 weeks due to rapid reaccumulation of pericardial fluid. A computed tomographic scan of the chest demonstrated no abnormality.
The patient was transferred from the neonatology intensive care unit to the cardiovascular surgery department at the ninth week of life for exploratory sternotomy. The pericardium was opened through median sternotomy. Approximately 150 mL of thin, yellowish fluid was aspirated. A partial adhesion of between the pericardium and the right atrial appendage was noted. Affected pericardium was easily dissected from the atrium and excised. The surface of the right atrial appendage and the free atrial wall appeared quite thick, firm, and included calcified areas, showing variegated colors ranging from white to dark red, which led to the consideration of the presence of a right atrial tumor. Biopsy was impossible because of the firm and thick structure of the lesion. Thus we made an on the spot decision to resect the tumor for diagnosis and to provide optimal treatment. Under cardiopulmonary bypass, moderate hypothermia, and cardioplegic arrest, the right atrium was opened. A standard dose of 20 mL/kg cardioplegia was administered. Spontaneous cardiac activity, however, was started just 5 minutes after this application. Three more doses had to be given during the cross-clamping period, which lasted a total of 18 minutes. An intramurally located tumor was observed that diffusely extended to the junction of the superior vena cava and atrioventricular groove. Complete excision was deemed impossible because of the extent of the tumor. The right atrial appendage and the free atrial wall were resected, and the defect was reconstructed with pericardium. The patient had an uneventful postoperative course, without any disturbance of the cardiac rhythm, and was discharged 7 days after the operation. Postoperative echocardiographic examination revealed some residual thickening of the right atrium at the superior vena caval junction. The patient is doing well 30 months after the operation. Serial electrocardiogram and echocardiographic examinations showed the presence of normal sinus rhythm and normal echocardiographic findings (Fig 1A,B,C).
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Fig 1. (A) Electrocardiographic rhythm of patients at the last follow-up. (B and C) Echocardiographic examination shows no residual tumor at age of 34 months. (LA = left atrium; RA = right atrium; SVC = superior vena cava.)
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Hematoxylin-eosin stained paraffin sections of the myocardial biopsy specimen revealed an infiltration of irregular vascular structures, separating the myocardial fibers (Fig 2). The tumoral cells had oval-spindle shaped hyperchromatic nuclei and scant or sometimes vacuolated cytoplasms (Fig 3). Cellular areas with minimal luminal formations and epithelioid groups were observed (Fig 4). Pleomorphism was mild and mitotic figures were rare. Necrosis was not noted. The silver impregnation method accentuates the vascular structures (Fig 5). Immunohistochemically, the neoplastic infiltration was positive for vimentin, CD31, and CD34 (Fig 6). An epithelioid hemangioendothelioma was diagnosed with positive surgical margins.
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Comment
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Epitheloid hemangioendothelioma is a member of a family of vascular tumors characterized by epithelioid endothelial cells, displaying a clinical behavior that is intermediate between that of epithelioid hemangioma and epithelioid angiosarcoma [2]. The clinical presentations, pathologic features, and biological behaviors of these tumors are different [2–4]. Epitheloid hemangioendothelioma has indolent behavior, although it may show significant mitotic activity and tumor necrosis with the potential to recur and metastasize even after long periods [3–4]. The origin of epitheloid hemangioendothelioma is a vessel, usually a medium-sized or large vein in the lung, liver, bone, and soft tissues [2]. It occurs in all age groups, although it rarely occurs in children with no sex predilection [4].
Hemangiomas may involve any part of the heart in the myocardium, endocardium, or the pericardium [5]. Right atrium is the most frequent site, and pericardial spreading with a nonhemorrhagic pericardial effusion is very common. These tumors have an unpredictable outcome and may involute, stop growing, or proliferate indefinitely [5].
Hemangiomas are usually asymptomatic. A great majority of hemangiomas appear as an autopsy finding. However, there are reported cases with pericardial effusions and arrhythmias that have lead to sudden death [5]. Dysfunctions of the atrioventricular valves, dyspnea on exertion, congestive heart failure, outflow obstruction, pseudoangina, and failure to thrive may also be present [6].
Although echocardiography is warranted for such patients, a firm diagnosis of the cardiac tumor may not be reached until surgical exploration followed up by pathologic examination. Rosai and colleagues [7] described an epitheloid hemoangioendothelioma of the left atrium, which was found incidentally during an open heart surgery for valvular replacement. Fetal echocardiography of our patient showed mild to moderate pericardial effusion but no additional cardiac abnormality. Pericardiocentesis immediately after delivery was followed by repeated echocardiographic examinations that yielded no definite findings to establish a firm diagnosis. This was possibly due to the diffuse nature of the tumor, with no apparent intracavitary mass. The definitive diagnosis was established by exploratory sternotomy, surgical excision, and microscopic examination of the specimen. Histologic findings of mitotic activity, cellular pleomorphism, and necrosis pointed to its malignant potential. Although our patient has shown no evidence of recurrence 30 months after surgery, a longer period of close follow-up is necessary due to the unpredictable prognosis of epitheloid hemoangioendothelioma of the heart.
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References
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- Novitzky D, Rose AG, Morgan JA, Barnard CN. Primary cardiac hemangiomas: a report of 2 cases. S Afr Med J. 1984;66:267–270[Medline]
- Weiss SW, Enzinger FM. Epithelioid hemangioendothelioma: vascular tumor often mistaken for a carcinoma. Cancer. 1982;50:970–981[Medline]
- de Nictolis M, Brancorsini D, Goteri G, Prat J. Epithelioid hemangioma of the heart. Virchows Arch. 1996;428:119–123[Medline]
- Marchino D, Fisher F, Hofstetter S. Epithelioid hemangioendothelioma of the heart with distant metastases: a case report and literature review. J Cardiovasc Surg. 1993;34:529–533[Medline]
- Lo LJ, Nucho RC, Allen JW, Rohde RL, Lau FYK. Left atrial cardiac hemangioma associated with shortness of breath and palpitations. Ann Thorac Surg. 2002;73:979–981[Abstract/Free Full Text]
- Brizard C, Latremouille C, Jebara VA, et al. Cardiac hemangiomas. Ann Thorac Surg. 1993;56:390–394[Abstract/Free Full Text]
- Rosai J, Gold J, Landy R. The histiocytoid hemangiomas: a unifying concept embracing several previously described entities of skin, soft tissue, large vessels, bone and heart. Hum Pathol. 1979;10:707–730[Medline]
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Abstract
Introduction
