Ann Thorac Surg 2002;74:914-916
© 2002 The Society of Thoracic Surgeons
Case report
Surgical excision of a hemangioendothelioma of the left ventricle
Hrvoje Gasparovic, MDa,
Darko Anic, MDa,
Dalibor Saric, MDb,
Vladimir Gasparovic, MD, PhD*b,
Zeljko Djuric, MDa,
Ivan Jelic, MD, PhDa
a Department of Cardiac Surgery, University Hospital Rebro, Zagreb, Croatia
b Department of Pediatrics and Medicine, University Hospital Rebro, Zagreb, Croatia
Accepted for publication April 1, 2002.
* Address reprint requests to Dr Gasparovic, Vinkoviceva 13, 10 000 Zagreb, Croatia
e-mail: hgasparovic{at}yahoo.com
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Abstract
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Primary intracardiac neoplasms are most commonly histologically benign with only 30% exhibiting signs of malignancy. Metastatic tumors of the heart are 30 times more common than primary cardiac tumors. Patients with intracardiac masses may present with dyspnea on exertion, thromboembolic events, pericarditis, arrhythmias, or congestive heart failure. In many, however, the diagnosis is made upon discovering a murmur in an otherwise asymptomatic patient. Hemangioendotheliomas are very rare tumors of the heart characterized by capillary-sized vessels lined by rounded and often multilayered endothelial cells. Cardiac muscle cells are found compressed between proliferating vascular channels. We present a patient with this unusual cardiac tumor with no previous cardiac history and a new systolic murmur.
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Introduction
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Cardiac hemangioendotheliomas are very rare vascular tumors. Symptoms are usually secondary to compression of cardiac structures, outflow tract obstructions, or thromboembolic events [1, 2]. Echocardiography focuses the diagnosis on an intracardiac mass. The clinical course of these neoplasms is intermediate between benign hemangiomas and malignant angiosarcomas. This report presents a case of a left ventricular hemangioendothelioma in a child. The tumor was resected with the aid of hypothermic cardiopulmonary bypass. Gross and microscopic findings as well as operative technique are discussed.
We report on a 7-month-old child admitted to the emergency department of an outside hospital because of fever. A physical examination revealed a 2/6 systolic murmur best heard at the apex of the heart with propagation towards the left axilla. An echocardiogram revealed a large intracardiac mass. She was found to have situs solitus, concordant atrioventricular and ventriculoarterial connections with no visible shunts. The semilunar as well as the atrioventricular valves were of normal morphology and function. The tumor measured 3 x 2 cm and was located in the left ventricle, in continuity with the anterolateral wall of the left ventricle (Fig 1).
It was well defined and spherical in shape. Turbulent flow was noted in the subaortic region with the tumor responsible for a 30-mmHg gradient across the left ventricular outflow tract. Cardiac catheterization was not performed. She was transferred to our institution where she was referred for surgical treatment of the intracardiac mass. She exhibited no signs of congestive heart failure nor were any thromboembolic events recorded. Her psychomotor development was normal for her age. Her electrocardiogram showed normal sinus rhythm with a heart rate of 150 beats per minute. The chest roentgenogram was unremarkable, save for a minimal increase in pulmonary vascular markings. Blood cultures were drawn repeatedly and were always found to be sterile. She was placed on vancomycin and ceftazidime empirically. Her fever was only temporarily alleviated by the standard antipyretic regimen using topical cooling and paracetamol. Pertinent laboratory values included a white blood cell count of 15.4 x 109/L and serum C-reactive protein concentration of 72 mg/L. Her hematocrit was 25 ml/dL with a room oxygen saturation of 92.8%. Her preoperative management included a transfusion of 100 ml of packed red blood cells. Even though an anemia was present, we found no evidence of thrombocytopenia, decreased fibrin or increased fibrin split products, which in combination with a hemangioendothelioma would be suggestive of Kasabach-Merritt’s syndrome [3]. The surgical procedure was performed through a median sternotomy. The patient was placed on cardiopulmonary bypass in the standard fashion with the superior and inferior vena cavae cannulated separately. A right atriotomy was made, followed by an incision in the atrial septum to gain access to the left atrium. We then inspected the tumor through the mitral valve. We concluded that the mass could not be safely evacuated through this approach. Hence, a left ventriculotomy was made. The tumor with its attachment to the lateral wall of the left ventricle was then clearly identified (Fig 2).
The mass was excised with a margin of left ventricular muscle adjacent to the attachment of the tumor. Following the closure of the ventriculotomy and the iatrogenic atrial septal defect, the cross-clamp was taken off. We then proceeded with the closure of the right atriotomy. The patient was taken off cardiopulmonary bypass uneventfully. The patient tolerated the procedure well and was transferred to the intensive care unit on dobutamine at 5 µg/kg/min. She was transferred to the step down unit on postoperative day 1 and her recovery was uneventful. Her fever had subsided on postoperative day 4 and did not recur. The histological analysis of the tumor indicated that the tumor was made up of numerous vascular channels lined by multilayered endothelial cells that obstructed some of these vascular structures. These findings were diagnostic of a hemangioendothelioma. Rare mitotic figures and some areas of necrosis were seen. We documented positive CD31, CD 34, and Factor VIII markers by immunohistochemical assays, which pointed to a vascular origin of the neoplasm (Fig 3).
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Fig 1. Preoperative echocardiogram demonstrating the tumor (TM) within the left ventricle (arrows). (LA = left atrium; RA = right atrium; RV = right ventricle.)
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Fig 3. Disorganized vascular channels lined by multilayered endothelium; immunohistochemical staining (CD31, x40).
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Comment
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Hemangioendotheliomas are vascular tumors most commonly found in the skin, liver, and spleen. They are rarely found as primary cardiac tumors. They are characterized by various degrees of endothelial proliferation within disorganized vascular canals. Macroscopically, they seem well defined, yet no distinct encapsulation is seen. They are considered to be borderline tumors, with their clinical course being intermediate between benign hemangiomas and malignant angiosarcomas [4, 5]. A consumptive coagulopathy secondary to platelet sequestration and destruction within the vascular lesion may develop, leading to microvascular thrombosis within the tumor. In the case we presented, an anemia was present but no evidence of a coagulopathy suggestive of Kasabach-Merritt’s syndrome was found. Symptoms most commonly associated with intracardiac masses are dyspnea on exertion, arrhythmias, congestive heart failure, thromboembolism, and pericarditis. Our patient presented with a fever, lacking any symptoms more specific to the heart. Blood, urine, and sputum cultures were drawn repeatedly and were always unremarkable. The fever subsided once the tumor was excised and it did not recur. With echocardiography becoming a more widespread tool for noninvasive analysis, cardiac tumors are presently detected at an earlier stage thereby increasing the potential for radical excision. In the case we presented, the tumor and its attachment were removed with the aid of hypothermic cardiopulmonary bypass. The postoperative echocardiogram demonstrated no residual tumor. The natural history of cardiac hemangioendotheliomas remains unpredictable. The reports in the literature are too scarce to provide substantial information on the long-term clinical behavior of these intracardiac neoplasms. We believe that regular echocardiographic follow-up examinations are mandatory in order to identify earlier any recurrences of these exceedingly rare vascular tumors.
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References
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- Manasse E., Nicolini F., Canziani R., Gallotti R. Left ventricular hemangioma. Eur J Cardiothorac Surg 1999;15:864-866.[Abstract/Free Full Text]
- Brizard C., Latremouille C., Jebara V.A., et al. Cardiac hemangiomas. Ann Thorac Surg 1993;56:390-394.[Abstract/Free Full Text]
- Kasabach H.H., Merritt K.K. Haemangioma with extensive purpura. Am J Dis Child 1940;59:1063-1070.[Abstract/Free Full Text]
- Robbins S.L., Cotran R.S. Pathologic basis of disease, 2nd ed. Philadelphia: W B Saunders, 1981:635.
- Rehring T.F., Deutchman A., Cross J.S. Polymorphous hemangioendothelioma. Ann Thorac Surg 1999;68:1396-1397.[Abstract/Free Full Text]
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Abstract
Introduction
