Eur J Cardiothorac Surg 1999;16:367-370
© 1999 Elsevier Science NL
Giant primary cardiac liposarcoma vascularized via the circumflex coronary artery
Christof Stamm,
Thomas Felderhoff,
Bernhard Herse,
Harald Dalichau
Department of Thoracic and Cardiovascular Surgery, Georg August Universität, Göttingen, Germany
Corresponding author. Children's Hospital Boston, Cardiac Surgery/Research, 300 Longwood Avenue, Boston, MA 02115, USA. Tel.: +1-617-355-6894; fax: +1-617-355-6742
e-mail: stamm_c{at}hub.tch.harvard.edu
 |
Abstract
|
|---|
A 51-year-old patient presented with a history of chest pain and progressive dyspnea. CT scan demonstrated a lipomatous mass encircling the heart and compressing the left lung. After median sternotomy, an intrapericardial tumor of 3200 g with a vascular pedicle arising from the circumflex coronary artery was resected. A histologic diagnosis of a well-differentiated liposarcoma was made. The patient is alive and well 2 years postoperatively. This tumor with its unusual vascularization is the largest of the few primary cardiac liposarcomas that have been reported.
Key Words: Cardiac tumors • Soft-tissue tumors • Cardiac malignancies
|
1. Introduction
|
|---|
Liposarcomas belong to the most common malignant soft-tissue tumors in adults, but primary cardiac liposarcomas are very rare. To the best of our knowledge, less than 20 cases have been reported. The treatment strategy depends on the origin, extension, and malignancy of the individual tumor. We describe the surgical treatment of a giant intrapericardial liposarcoma that completely encircled the heart and the great vessels, originated from the left atrium, and derived its blood supply from a large tumor vessel originating from the circumflex coronary artery.
|
2. Case report
|
|---|
A 51-year-old woman presented with progressive dyspnea, fever, and night sweats. She had a 10-year history of left-sided chest pain, but heart disease had been excluded by previous evaluations and the complaints had initially been attributed to obesity and anxiety. Clinical examination revealed no clear signs of cardiac tamponade such as distended neck veins or liver enlargement. ECG showed low voltage but no axis shift. Chest X-rays demonstrated a markedly enlarged heart silhouette (heart/thorax ratio 21/28 cm) with opacification of the left hemithorax (Fig. 1). There were no signs of pulmonary congestion. Echocardiography demonstrated normally sized ventricles with good global contractility, although some areas of the left ventricular free wall appeared hypokinetic. Laboratory tests showed signs of inflammation (erythrocyte sedimentation rate >100 mm/h, fibrinogen 803 mg/dl, CRP 176 mg/dl, LDH 300 units/l). Computed tomography demonstrated a giant lipomatous tumor in the anterior mediastinum (Fig. 2). The upper mediastinum and the heart were displaced to the right, and the left lung was compressed with a completely atelectatic lower lobe. No evidence of metastases was apparent. Since intrapericardial tumor localization was not suspected, coronary angiography was not performed. The patient was referred to the Department of Thoracic and Cardiovascular Surgery for resection of the tumor. After median sternotomy and incision of the pericardium a giant intrapericardial lipomatous tumor was encountered. The tumor appeared to originate from the left atrial appendage but a vascular pedicle arose from the circumflex coronary artery. The heart and the great vessels were completely encircled by the tumor mass. Separation from the left and right ventricular surface was difficult due to multiple adhesions between the tumor and the epicardium, and infiltrative growth was suspected. The tumor was separated from the epicardium mainly by blunt dissection. Sharp dissection, when necessary, was performed carefully to avoid damage to coronary arteries. The pericardium was not resected to avoid distortion of or tension on the left phrenic nerve. Several plicating sutures were used to decrease the excessive intrapericardial space. A large tumor vessel originated from the circumflex coronary artery and could be detached without interrupting the continuity of the coronary vessel. Eventually, complete resection of the neoplasm was accomplished without cardiopulmonary bypass or cardiac arrest. The tumor weight was 3200 g. Histologic examination revealed a neoplasm composed of fatty tissue with focal necroses, and the fat vacuoles varied markedly in size. Immunohistochemistry using antibodies against the proliferation marker MIB1 demonstrated nuclear staining of fat cells. In the adjacent ventricular tissue, myocardial fibers were compressed, displaced and separated by proliferating tumor cells. The final histological diagnosis of a low grade, well-differentiated liposarcoma (ICD-O-8851/3) was made.
View larger version (109K):
[in this window]
[in a new window]
|
Fig. 1. Preoperative chest X-ray showing a globally enlarged heart silhouette with an atelectatic left lower lobe.
|
|
View larger version (139K):
[in this window]
[in a new window]
|
Fig. 2. CT scan demonstrating a hypodense tumor mass encircling the heart. The arrowhead points to a large tumor vessel originating from the circumflex coronary artery.
|
|
The early postoperative course was complicated by symptoms of global heart failure. The patient required mechanical ventilation for 3 days and 5 days of treatment with catecholamines until satisfactory cardiac recompensation was achieved. The further hospital course was uneventful and the patient discharged 14 days postoperatively. Two years after surgery the patient is alive and well with no clinical or radiological signs of local recurrence or metastatic disease.
|
3. Comments
|
|---|
Liposarcomas are the second-most frequent soft-tissue tumors in adults [1,2]. Enzinger and Winslow have described well differentiated (lipoma-like), myxoid, round cell, and pleomorphic types [6]. This histological classification correlates well with the prognosis. Local expansion is typical for well-differentiated and myxoid liposarcomas, and 5-year survival after resection is almost 80%. Round cell and pleomorphic liposarcomas are aggressive tumors that are characterized by infiltrative growth, early metastasis to the lungs as well as frequent local recurrence after surgical resection. Five-year survival rates vary between 25 and 50%.
Primary cardiac liposarcomas are extremely rare. The majority originate from the right heart, particularly from the right atrium, but some reports describe tumors originating from the mitral valve [3], the pulmonary veins [4], or the aortic root [5]. Similar to other tumors of the heart, most cardiac liposarcomas remain undiagnosed until symptoms of cardiac constriction develop. Usually chest pain, dyspnea, dysrhythmia, or signs of congestive heart failure or arterial embolism are observed. Cancer-associated symptoms such as weight loss or night sweats develop later on in the course of the disease [7]. The treatment of choice is radical excision, and several cases of repeated excision for recurrent cardiac liposarcomas with good patient survival have been reported [4,8]. Radiotherapy is utilized as adjuvant treatment in cases of poorly differentiated tumors, incomplete resection or inoperability, since liposarcomas are considered to be insensitive to chemotherapy [1,9].
We present this case of a primary cardiac liposarcoma because of its remarkable size (the largest reported so far) and unusual vascular origin. The lack of symptoms indicating cardiac constriction may be explained by the slow growth of the tumor. However, it can not be excluded that some of the patient's long-standing complaints were initially misinterpreted. Despite the extension of the tumor and superficial infiltration of the left ventricular surface it could be completely resected, so that adjuvant radiotherapy was not indicated. This large tumor derived its blood supply from the circumflex coronary artery, but it remains unclear whether this contributed to the preoperative symptoms. Based on our experience with this case we recommend preoperative coronary angiography whenever a comparable mediastinal is encountered. The hospital course was complicated by global heart failure, which is frequently observed after relief of a chronic cardiac constriction. Similar to patients who undergo total pericardectomy for constrictive pericarditis, a temporary biventricular dilation can develop that usually resolves after several days of inotropic support.
|
References
|
|---|
-
Springfield D. Liposarcoma. Clin Orthop 1993;289:50-57.
-
Morton D.L., Antman K.H., Tepper J. Soft tissue sarcomas. In: Holland J.F., ed. Cancer Medicine. vol. 2. 1993:1862-1863.
-
Paraf F., Bruneval P., Balaton A., Deloche A., Mikol J., Maitre F., Scholl J.M., DeSaint-Maur P.P., Camilleri J.P. Primary liposarcoma of the heart. Am J Cardiovasc Pathol 1990;3(2):175-180.[Medline]
-
Baubion N., Block E., Donne C., Coudurier P., Jaubert F., Metzger J.P., Heulin A., Vacheron A. Lipomyxosarcoma of the pulmonary veins extending into the left atrium. Repetitive surgical treatment. Presse Med 1985;14(5):267-269.
-
Lacey C.J., Petch M.C. Primary liposarcoma of the pericardium. Thorax 1979;34(1):120-122.[Medline]
-
Enzinger F.H., Winslow D.J. Liposarcoma. A study of 103 cases. Virchows Arch Pathol Anat 1962;355:367-388.
-
Silverman N.A. Primary cardiac tumors. Ann Surg 1980;191(2):127-138.[Medline]
-
Kendall S.W., Williams E.A., Hunt J.B., Petch M.C., Wells F.C., Milstein B.B. Recurrent primary liposarcoma of the pericardium: management by repeated resections. Ann Thorac Surg 1993;56(3):560-562.[Abstract]
-
Llombart-Cussac A., Pivot X., Contesso G., Rhor-Alvarado A., Delord J.P., Spielmann M., Tursz T., LeCesne A. Adjuvant chemotherapy for primary cardiac sarcomas: the IGR experience. Br J Cancer 1998;78(12):1624-1628.[Medline]
Received May 17, 1999;
received in revised form July 12, 1999;
accepted July 13, 1999.
This article has been cited by other articles:
|
|
|
|
 
S. C. Gaerte, C. A. Meyer, H. T. Winer-Muram, R. D. Tarver, and D. J. Conces Jr
Fat-containing Lesions of the Chest
RadioGraphics,
October 1, 2002;
22(90001):
S61 - 78.
[Abstract]
[Full Text]
[PDF]
|
|
|
Top
Abstract
1. Introduction
