Asian Cardiovasc Thorac Ann 1999;7:52-55
© 1999 Asia Publishing EXchange Pte Ltd
Cardiac Sarcomas: Is Tumor Debulking Justifiable Therapy?
Reshma Manoj Biniwale, MCh,
Hemant Pramod Pathare, MCh,
Nandkumar Aggrawal, MCh,
Anil Gangadhar Tendolkar, MS,
Jaya Deshpande, MD,1,
Amoo Sivaraman, MD,1
Department of Cardiovascular & Thoracic Surgery India
1 Department of Cardiovascular Pathology King Edward VII Memorial Hospital Mumbai, India
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For reprint information contact: Hemant Pramod Pathare, MCh Tel: 91 22 611 3358 Fax: 91 22 611 5958 6 Queen's Croft CHS, St. Xavier's School Road, Ville-Parle (West), Mumbai 400056, India.
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Abstract
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During a 15-year period from 1982 to 1997, 4 cases out of approximately 12,000 open-heart operations were performed at our institute for malignant cardiac tumors. Three of these tumors were angiosarcomas and one was a high-grade spindle-cell sarcoma. The prognosis for all 4 patients was uniformly poor in spite of maximal tumor debulking and postoperative chemotherapy.
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Introduction
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Cardiac sarcomas are extremely rare malignant tumors of mesodermal origin. Any of the cardiac chambers may be involved. Such tumors are very aggressive and usually incurable at the time of diagnosis.
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Case Reports
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We describe our experience of 4 cases of cardiac sarcoma that we encountered in a 15-year period from 1982 to 1997 among approximately 12,000 open-heart surgical cases at King Edward VII Memorial Hospital, Mumbai, India. All 4 patients were male adults aged between 22 and 45 years. Cardiac catheterization and angiography were used for definitive diagnosis in 3 patients (between 1983 and 1984).
Case 1
A 22-year-old male presented with a 3-month history of palpitations, non-anginal precordial chest pain, and nonproductive cough accentuated in the right lateral decubitus position. Six weeks previously, he had been investigated in another center where a chest radiograph and a two-dimensional echocardiogram were normal but his electrocardiogram revealed sinus tachycardia, T-wave inversion in leads V2 to V6, and occasional ventricular ectopic beats. Since then, his weight had decreased by 7 kg. On admission to our institute, he had tachycardia and hypotension. The neck veins were not engorged. Chest radiography showed cardiomegaly (cardiothoracic ratio, 0.65) with superior mediastinal widening (Figure 1
). An electrocardiogram showed sinus rhythm, a normal PR interval, and a left ventricular strain pattern. Two-dimensional echocardiography revealed an anterior mediastinal mass causing compression of the right ventricular outflow tract and a left ventricular ejection fraction of 60%. A computed tomography scan of the chest confirmed the presence of an anterior mediastinal mass invading the left hemidiaphragm inferiorly and extending up to the anterolateral cardiac border laterally (Figure 2). The appearance of the mass was suggestive of a thymoma or lymphoma. Fiberoptic bronchoscopy showed indentation of the trachea and compression of the left main bronchus. A computed tomography-guided needle biopsy of the mass was attempted twice but only necrotic material and blood was obtained. Laboratory tests were unhelpful. Exploration was undertaken via a left anterolateral thoracotomy. The lungs and chest wall were free of tumor tissue and there was minimal pleural effusion. The pericardium and the left cardiophrenic nerve were stretched over an intrapericardial mass. There was 600 mL of hemorrhagic pericardial effusion and a large irregular left ventricular tumor. With cardiopulmonary bypass equipment on standby, multiple biopsies were collected and the mass was debulked. Frozen section histology suggested undifferentiated sarcoma. Hemostasis was achieved with difficulty using a pericardial patch and oxidized cellulose (Johnson and Johnson, Arlington, TX, USA). A single 32 F tube inserted in the left pleural cavity drained approximately 1800 mL of blood over the next 48 hours, which was replaced. Histopathology revealed a high-grade spindle-cell sarcoma (Figure 3). The patient received postoperative chemotherapy consisting of ifosfamide and cisplatin. However, he developed cachexia and local recurrence of the tumor and died 3 months later.
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Figure 1. Radiograph (posteroanterior view) of a patient with left ventricular sarcoma, demonstrating cardiomegaly with a cardiothoracic ratio of 0.65 (case 1).
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Figure 3. Histopathological appearance of the high-grade spindle-cell sarcoma of the left ventricle (case 1). Fascicles of malignant pleomorphic spindle cells can be seen. Note the mitotic figures (arrows) (hematoxylin and eosin stain, original magnification x400).
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Case 2
A 45-year-old male presented with chest pain, exertional dyspnea, fatigue, and weakness of 6 months duration. His pulse rate was 80 beats•min–1, blood pressure 120/80 mm Hg, jugular venous pressure was elevated with prominent a and v waves, and he had hepatomegaly. Cardiovascular examination showed cardiomegaly, a loud S3 gallop, and a grade 2/6 pansystolic murmur along the left sternal border. Chest radiography showed a cardiothoracic ratio of 0.65 and a prominent left cardiac border. An electrocardiogram revealed sinus rhythm and ST–T changes in leads V1 to V6. Hemodynamic studies indicated elevated ventricular diastolic pressure, a systolic gradient across the right ventricular outflow tract, and suspicion of cardiac compression. Right ventricular angiography disclosed distortion of the cavity, mild tricuspid regurgitation, and difficulty in visualizing the right ventricular outflow tract. A left ventricular angiogram was normal. Dye injection into the left coronary artery demonstrated displacement of the left anterior descending artery. There was no tumor blush. Selective right coronary artery angiography was normal. Exploration under cardiopulmonary bypass revealed a large tumor occupying the right atrium and invading the overlying pericardium. It was difficult to ascertain the extent of the tumor and the approachable section was excised. Tumor histopathology revealed angiosarcoma.1 Postoperative hemodynamic studies showed a mild residual gradient across the right ventricular outflow tract with no distortion of the right ventricular cavity or outflow tract. Postoperatively, the patient had adjuvant chemotherapy consisting of adriamycin and dacarbazine. He survived for 8 months.
Case 3
A 24-year-old male was admitted with class II dyspnea on exertion and cough with hemoptysis. He was febrile and had marked pallor. His jugular venous pressure was raised with prominent a and v waves and his systemic pressure was 120/80 mm Hg. There was a dull note on percussion in the right paracardiac region. A mid-diastolic murmur was heard in the tricuspid area and it increased on inspiration. The liver was palpable 3 cm below the costal margin. Chest radiography showed a paracardiac mass in continuity with the cardiac shadow, which was non-pulsatile on fluoroscopy. There was no cardiomegaly and the lung fields were clear. His electrocardiogram and M-mode echocardiogram were normal. Aortography revealed a vascular mass fed by several vessels from the right subclavian artery, internal mammary artery, inferior phrenic and lateral thoracic arteries, suggesting an angiomatous neoplasm. The patient discharged himself against medical advice. Three months later, he returned with chest pain and increased breathlessness. He was found to have right hemothorax. Intercostal drainage produced 600 mL of fresh blood and the patient underwent an emergency right lateral thoracotomy. This disclosed the extent of the tumor to be 9 x 5.5 x 3.5 cm so a median sternotomy incision was required. A large vascular mass was seen extending posteriorly from the right border of the heart into the hilum of the right lung. In view of the vascularity and involvement of the right atrium, profound hypothermia and total circulatory arrest were established. The entire visible mass was excised along with part of the right atrial wall as a means of debulking the tumor. Postoperatively, the patient lapsed into disseminated intravascular coagulopathy and could not be salvaged. Histopathology of the tumor revealed multiple slit-like spaces surrounded by small spindle-shaped cells with dark nuclei and scant cytoplasm, suggestive of angiosarcoma.2 At autopsy, the heart was seen to be moderately enlarged and a partly cystic and partly solid residual mass of 4 x 3 x 1.5 cm was found in the right atrial cavity, extending into the interatrial septum and partially obstructing the tricuspid valve, which was normal. The left lung contained multiple subpleural nodules.
Case 4
A 45-year-old male was referred with progressive cardiomegaly following an episode of chest pain 4 months previously. Chest radiography showed a cardiothoracic ratio of 0.6 with a hump on the left heart border. The electrocardiogram showed inverted T waves in lead 1, aVL, and the lateral chest leads. A coronary angiogram was normal. A right ventricular angiogram showed a filling defect, gross tricuspid regurgitation, and failure of the dye to enter the main pulmonary artery (Figure 4 ). The patient was diagnosed with an intrapericardial mass and explored through a median sternotomy. On opening the pericardium, a large soft fleshy friable mass was seen around the ventricles with no discernible plane between the mass and the heart. The mass was excised without using cardiopulmonary bypass, leaving only a thin layer over the heart. The pericardium was left open. Histopathology revealed angiosarcoma. The patient received adjuvant chemotherapy with adriamycin and dacarbazine. Angiography 6 months later showed normal filling of the right ventricle and main pulmonary artery (Figure 5). The tricuspid regurgitation was markedly reduced. He survived for another 12 months on adjuvant chemotherapy.
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Figure 4. Right ventricular angiogram showing tricuspid regurgitation, right ventricular cavity distortion, and failure to visualize the outflow tract and main pulmonary artery (case 4). Reproduced with permission from Mosby Inc. (Am Heart J 1985;109:601–3).
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Figure 5. Postoperative right ventricular angiogram (posteroanterior view) showing normal opacity of the right ventricular cavity and outflow tract, including the main pulmonary arterial trunk (case 4). Reproduced with permission from Mosby Inc. (Am Heart J 1985;109:601–3).
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Discussion
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The specific symptoms and signs produced by cardiac tumors are more closely related to their anatomical location than to their histological type. Almost all malignant cardiac tumors are sarcomas, including angiosarcoma, rhabdomyosarcoma, osteosarcoma, and fibrosarcoma Sarcomas are the second most common cardiac tumors after myxomas. Myocardial tumors commonly result in arrhythmias and rarely in conduction block. Impairment of ventricular performance may simulate congestive, restrictive, or hypertrophic cardiomyopathy. Sarcomatous tumor infiltration of the myocardium may occasionally cause myocardial rupture. Patients with right ventricular sarcomas often present with right heart failure due to obstruction of right ventricular filling or outflow.3 Tumor emboli in the pulmonary arteries may result in pulmonary hypertension. Left ventricular tumors may be associated with conduction disturbances or outflow tract obstruction with syncope, heart failure, and atypical chest pain.4
Sarcomas grow rapidly and may cause hemorrhagic pericardial effusion or obliteration of the pericardial space resulting in tamponade, local mediastinal invasion, and intramural or intracavitary extension. Malaise, fever, and fatigue are usually seen before signs of outflow tract obstruction, tamponade, or conduction disturbances.4 Sarcomas cause rapid clinical deterioration over a few weeks or up to 2 years after diagnosis due to widespread infiltration of the myocardium and intracavitary spread, ventricular outflow tract obstruction, or metastases to the lungs, mediastinal lymph nodes, and vertebrae. Angiosarcomas most commonly arise from the right atrium and involve the overlying pericardium. Metastasis has been reported in 66% to 89% of patients with mean survivals of 3 to 6.6 months.5,6
In our patients, the most common presenting symptom was chest pain (in 3 cases), followed by exertional dyspnea, easy fatigability, and significant weight loss (in 2 cases each). No patient suffered an episode of syncope. Cardiomegaly on chest radiographs was a consistent feature. Arrhythmias in relatively young patients may suggest the possibility of a cardiac tumor and when the diagnosis is suspected preoperatively, an endomyocardial biopsy may be carried out for confirmation. Being such rare entities, cardiac sarcomas are most often misdiagnosed as mediastinal masses. The major role of surgery is to establish a diagnosis and exclude the possibility of a curable benign tumor. Limited palliation and extension of life may be achieved by aggressive debulking along with adjuvant chemotherapy or radiotherapy. Radical resection may result in severe cardiac functional impairment and death.
After observing the uniformly poor prognoses of our patients after aggressive debulking and postoperative chemotherapy, and the poor quality of life thereafter, we concluded that it was not justified to subject such compromised patients to major surgery. Palliative cardiomyoplasty was reported by Carpentier and Chachques7 in a patient with a cardiac tumor that involved most of the left ventricle. Although technically successful cardiac transplantation for patients with angiosarcomas has been reported, this option is limited; the maximal survival was 8 to 9 months and the authors concluded that malignant cardiac tumors are a contraindication to transplantation.8
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Acknowledgments
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We thank Girish Moghe, MD, Department of Pathology, Tata Memorial Hospital, Mumbai, for his assistance in preparing this report and also Pragnya Pai, MD, Dean, King Edward VII Memorial Hospital, Mumbai, for allowing us to use the hospital data.
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References
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Sharma S, Tendolkar AG, Parulkar GB. Angiosarcoma of the heart. Am Heart J 1985;109:601–3.[Medline]
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Tendolkar AG, Joshi SV, Parulkar GB. Angiosarcoma of the heart. Indian Heart J 1984;36:188–91.[Medline]
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Bojar R, editor. Malignant cardiac tumours. Adult cardiac surgery. Boston: Blackwell Scientific, 1992:366–71.
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Glancy DL, Morales JB Jr, Roberts WC. Angiosarcoma of the heart. Am J Cardiol 1968;21:413–9.[Medline]
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McFadden PM, Ochsner JL. Atrial replacement and tricuspid valve reconstruction after angiosarcoma resection. Ann Thorac Surg 1997;64:1164–6.[Abstract/Free Full Text]
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Janigan DT, Husain A, Robinson NA. Cardiac angio-sarcomas: a review and a case report. Cancer 1986;57:852–9.[Medline]
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Carpentier A, Chachques JC. Myocardial substitution with a stimulated skeletal muscle: first successful case. Lancet 1985;1:1267.[Medline]
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Crespo MG, Pulpon LA, Pradas G, Serrano S, Segovia J, Vegazo I, et al. Heart transplant for cardiac angiosarcoma: should its indication be questioned? J Heart Lung Transplant 1993;12:527–30.[Medline]
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Abstract
Introduction
