DOI: 10.1148/rg.23si035140
(Radiographics. 2003;23:S141-S145.)
© RSNA, 2003
Best Cases from the AFIP
Cardiac Angiosarcoma1
Andrew K. Best, MD,
Rebecca L. Dobson, MD, FRCPC and
Asma R. Ahmad, MD, FRCPC
1 From the Queen Elizabeth II Health Sciences Centre, 1278 Tower Rd, Victoria General Site, Halifax, Nova Scotia, Canada, B3H 2Y9. Received June 4, 2003; revision requested July 1 and received July 23; accepted July 28. Address correspondence to A.B. (e-mail: abest@is2.dal.ca).
Index Terms: Heart, neoplasms, 521.324 • Sarcoma, 521.324
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History
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A 30-year-old male truck driver presented to a community hospital with increasing shortness of breath. At physical examination, he was found to be in respiratory distress and had a pulsus paradoxus, a finding suggestive of cardiac tamponade. A total of 900 mL of bloody pericardial fluid was aspirated. The patient was stabilized, was transferred to our institution, and underwent echocardiography, which showed a homogeneous mass that involved the free wall of the right atrium. The patient then underwent computed tomography (CT), magnetic resonance (MR) imaging, and angiography. Findings from endovascular biopsy of the mass suggested a diagnosis of cardiac angiosarcoma. At surgery, the tumor, as well as part of the right pericardium, were resected. A piece of bovine pericardium was used to reconstruct the right atrial wall. The patient was discharged home in good condition, but repeat CT of the thorax performed only 7 weeks later showed lung and pericardial metastases. Cranial CT performed 2
months after the initial surgery showed brain metastases.
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Imaging Findings
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At transthoracic echocardiography, a right atrial mass that occupied one-third of the right atrial chamber was seen. The tricuspid valve was not involved. Contrast material–enhanced CT showed a low-attenuation mass arising from the anterior wall of the right atrium with no evidence of lung metastases (Fig 1a). Cardiac MR imaging demonstrated an enhancing mass that involved the right atrial wall from the upper midportion and extended down to the floor of the right atrium (Fig 1b, 1c). There was no involvement of the superior or inferior venae cavae. Cardiac angiography revealed a large atrial collateral vessel arising from the right coronary artery that supplied neovasculature in the region of the mass (Fig 1d).
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Figure 1a. Cardiac angiosarcoma. (a) Axial contrast-enhanced CT scan shows a low-attenuation, lobulated right atrial mass (arrow). (b) Axial T1-weighted MR image obtained with gadolinium shows nodular enhancement of the right atrial mass (arrow). (c) Coronal T1-weighted MR image obtained without gadolinium shows the mass (arrow) arising from the free wall of the right atrium. The mass is slightly hyperintense relative to myocardium. (d) Right coronary angiogram shows tumor blush (arrow).
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Figure 1b. Cardiac angiosarcoma. (a) Axial contrast-enhanced CT scan shows a low-attenuation, lobulated right atrial mass (arrow). (b) Axial T1-weighted MR image obtained with gadolinium shows nodular enhancement of the right atrial mass (arrow). (c) Coronal T1-weighted MR image obtained without gadolinium shows the mass (arrow) arising from the free wall of the right atrium. The mass is slightly hyperintense relative to myocardium. (d) Right coronary angiogram shows tumor blush (arrow).
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Figure 1c. Cardiac angiosarcoma. (a) Axial contrast-enhanced CT scan shows a low-attenuation, lobulated right atrial mass (arrow). (b) Axial T1-weighted MR image obtained with gadolinium shows nodular enhancement of the right atrial mass (arrow). (c) Coronal T1-weighted MR image obtained without gadolinium shows the mass (arrow) arising from the free wall of the right atrium. The mass is slightly hyperintense relative to myocardium. (d) Right coronary angiogram shows tumor blush (arrow).
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Figure 1d. Cardiac angiosarcoma. (a) Axial contrast-enhanced CT scan shows a low-attenuation, lobulated right atrial mass (arrow). (b) Axial T1-weighted MR image obtained with gadolinium shows nodular enhancement of the right atrial mass (arrow). (c) Coronal T1-weighted MR image obtained without gadolinium shows the mass (arrow) arising from the free wall of the right atrium. The mass is slightly hyperintense relative to myocardium. (d) Right coronary angiogram shows tumor blush (arrow).
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Pathologic Evaluation
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Initial examination of the specimen obtained at endovascular biopsy revealed tumor tissue with vascular channels. These channels were lined with plump endothelial cells demonstrating nuclear atypia and pleomorphic nuclei, some of which contained nucleoli. There was positive reactivity of the tumor cells with CD31 and CD34, which are immunohistochemical markers for endothelial cells. Electron microscopic findings were also consistent with those of a tumor of endothelial origin.
The resected tumor from the anterior right atrial wall measured 4.0 x 3.0 x 3.0 cm. The external surface of the tumor was nodular and covered in hemorrhagic and necrotic material (Fig 2).
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Figure 2a. Cardiac angiosarcoma. (a) Photograph of the resected specimen shows a portion of the right atrium and intraatrial tumor. Arising from the free wall of the right atrium, the angiosarcoma shows frondlike papillary projections (arrow). (b) Photograph of the bisected angiosarcoma shows areas of hemorrhage and necrosis (arrow).
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Figure 2b. Cardiac angiosarcoma. (a) Photograph of the resected specimen shows a portion of the right atrium and intraatrial tumor. Arising from the free wall of the right atrium, the angiosarcoma shows frondlike papillary projections (arrow). (b) Photograph of the bisected angiosarcoma shows areas of hemorrhage and necrosis (arrow).
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Sections of the right atrial tumor consisted of a large, partly necrotic and hemorrhagic vascular tumor (Fig 3). There were focal areas of papillary configuration and dense areas with tight cells of endothelial origin surrounding small capillary channels. Individual cells showed eosinophilic cytoplasm and contained pleomorphic nuclei with round, oval, and spindle-shaped configuration. A few bizarre and multinucleated forms were also identified. The mitoses were numerous, with the count being higher than 15 mitotic figures per high-power field. The tumor involved the full thickness of the atrial wall and also invaded the right ventricular wall. The overall morphology of the tumor was consistent with a high-grade invasive cardiac angiosarcoma.
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Figure 3a. Cardiac angiosarcoma. (a) Photomicrograph (original magnification, x4; hematoxylin-eosin stain) shows frondlike projections (arrow) arising from the luminal surface of the angiosarcoma. In this particular section, the right atrial wall is completely replaced by tumor, necrosis, and hemorrhage. (b) Low-power photomicrograph (original magnification, x40; hematoxylin-eosin stain) shows the angiosarcoma (arrows) invading normal right atrial myocardium. (c) Photomicrograph (original magnification, x100, hematoxylin-eosin stain) shows red blood cells clustered together within a vascular channel (arrow) lined with atypical endothelial cells. This appearance is characteristic for angiosarcoma.
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Figure 3b. Cardiac angiosarcoma. (a) Photomicrograph (original magnification, x4; hematoxylin-eosin stain) shows frondlike projections (arrow) arising from the luminal surface of the angiosarcoma. In this particular section, the right atrial wall is completely replaced by tumor, necrosis, and hemorrhage. (b) Low-power photomicrograph (original magnification, x40; hematoxylin-eosin stain) shows the angiosarcoma (arrows) invading normal right atrial myocardium. (c) Photomicrograph (original magnification, x100, hematoxylin-eosin stain) shows red blood cells clustered together within a vascular channel (arrow) lined with atypical endothelial cells. This appearance is characteristic for angiosarcoma.
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Figure 3c. Cardiac angiosarcoma. (a) Photomicrograph (original magnification, x4; hematoxylin-eosin stain) shows frondlike projections (arrow) arising from the luminal surface of the angiosarcoma. In this particular section, the right atrial wall is completely replaced by tumor, necrosis, and hemorrhage. (b) Low-power photomicrograph (original magnification, x40; hematoxylin-eosin stain) shows the angiosarcoma (arrows) invading normal right atrial myocardium. (c) Photomicrograph (original magnification, x100, hematoxylin-eosin stain) shows red blood cells clustered together within a vascular channel (arrow) lined with atypical endothelial cells. This appearance is characteristic for angiosarcoma.
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Discussion
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Epidemiologic Features
Cardiac sarcomas are the most common primary malignant tumor of the heart and pericardium (1). They are rare neoplasms, with a prevalence of 0.001%–0.3% in autopsy series (2). It is worth noting that cardiac metastases are 20–40 times more common than primary cardiac tumors (3). Even among primary cardiac tumors, the majority (approximately 75%) are benign and most frequently are atrial myxomas (4).
There are several subtypes of cardiac sarcoma, with angiosarcoma being the most common in adults (1). Other subtypes include rhabdomyosarcoma, malignant fibrous histiocytoma, undifferentiated, and fibrosarcoma.
No more than 300 cases of cardiac angiosarcoma have been reported in the literature worldwide. Cardiac angiosarcomas can occur in patients of any age group but are most commonly found in 30–50-year-old patients (1,5). There is a male-to-female predominence of about two to one.
Clinical Features
Unfortunately, the signs and symptoms of cardiac angiosarcoma are entirely nonspecific, and the diagnosis may not be made until surgery or even autopsy. Presenting complaints include chest pain, shortness of breath, malaise, or fever. At physical examination, the patient may have hypotension and tachycardia. Cardiac auscultation may reveal diminished heart sounds or a friction rub. More than 75% of patients have electrocardiographic abnormalities, but again these findings are protean and include arrhythmias, heart block, and nonspecific ST and T wave changes (6).
Cardiac tamponade from a bloody pericardial effusion may lead to heart failure, as occurred in our case. Apparently, this event commonly leads to patient presentation in cases of cardiac angiosarcoma (7).
Imaging Features
Nearly 80% of cardiac angiosarcomas arise as mural masses in the right atrium. They may invade adjacent structures such as the superior or inferior venae cavae or the tricuspid valve.
Echocardiography remains the initial imaging modality of choice for evaluating cardiac masses. It accurately depicts cardiac anatomy in multiple planes as well as involvement of the tricuspid valve. However, echocardiography has limited ability to demonstrate tumor infiltration and cannot depict mediastinal and extracardiac involvement.
The most common chest radiographic finding is cardiomegaly (1). Other abnormalities include right-sided heart enlargement, widened mediastinum, hilar adenopathy, pulmonary congestion, or pleural effusion.
At cross-sectional imaging studies, cardiac angiosarcomas demonstrate two patterns of growth. The first and more common pattern is a well-defined mass arising from the free wall of the right atrium (5). Often, these masses contain areas of hemorrhage and necrosis, which appear with a heterogeneous pattern on MR images (8). Areas of high signal intensity within the tumor are thought to represent subacute blood products (9). CT usually shows a low-attenuation right atrial mass, which may be irregular or nodular (10). Enhancement with contrast material is typically heterogeneous on both CT and MR images.
The second and less common pattern of growth involves tumor infiltration along the pericardium, in which pericardial effusion and thickening may be seen on CT scans.
Differential Diagnosis
Given the nonspecific clinical presentation, the differential diagnosis is very broad. It includes coronary artery disease, pulmonary embolism, pericarditis, constrictive cardiomyopathy, and other noncardiac malignancies such as bronchogenic carcinoma or mesothelioma.
Histologic Diagnosis
Angiosarcoma is a tumor of endothelial and mesenchymal cells (10). They are usually hemorrhagic and often have poorly defined borders. They are aggressive tumors, often invading contiguous structures such as the venae cavae and tricuspid valve. The vast majority arise from the right atrium (2,5,7,8). Cardiac angiosarcomas can both invade and encase the myocardium. Characteristic areas of vascular channels lined by pleomorphic and atypical cells are seen. The presence of irregular anastomosing sinusoidal structures with papillary intraluminal tufting is diagnostic of these tumors (7). Higher mitotic rates and necrosis are correlated with poorer outcome (1). Typically, these tumors stain positive for a variety of immunohistochemical markers, including CD34, CD31, factor VIII–related antigen, and vimentin. The hallmark of cardiac angiosarcoma at electron microscopy is Weibel-Palade bodies, which are characteristic for endothelial cells, but these structures are often not seen (11).
Treatment
No conclusive strategy exists for treating cardiac soft-tissue tumors. The mainstay of treatment is surgical resection (4). Unfortunately, patient outcome is almost invariably poor because of the unfavorable location of these tumors, their rapid growth, and early metastases. However, surgical excision can substantially alleviate symptoms and yield a modest improvement in survival. The mean survival time without surgical resection is approximately 4 months, which improves to 10 months following surgery (5).
The role of chemotherapy and irradiation is evolving, but traditionally angiosarcomas are thought to be unresponsive to these treatments (7). Although heart transplantation has been attempted, the results remain disappointing (11).
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Conclusions
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Cardiac angiosarcoma is a rare diagnosis and one that is often difficult to make because of the nonspecific presenting signs and symptoms. Initial diagnosis is usually suggested at echocardiography, but identification of mediastinal invasion and extracardiac metastases is best achieved with CT and MR imaging. The overall prognosis remains poor despite advances in surgical techniques. There is, however, an encouraging trend toward more accurate and earlier diagnosis, in large part because of improved imaging techniques.
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Footnotes
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Editor’s Note.—Everyone who has taken the course in radiologic pathology at the Armed Forces Institute of Pathology (AFIP) remembers bringing two beautifully illustrated cases for accession to the Institute. In recent years, the staff of the Department of Radiologic Pathology has judged the "best cases" by organ system, and recognition is given to the winners on the last day of the class. With each issue of RadioGraphics, one or more of these cases are published, written by the winning resident. Radiologic-pathologic correlation is emphasized, and the causes of the imaging signs of various diseases are illustrated.
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References
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- Burke AP, Cowan D, Virmani R. Primary sarcomas of the heart. Cancer 1992; 69:387-395.[CrossRef][Medline]
- Glancy DL, Morales JB, Roberts WC. Angiosarcoma of the heart. Am J Cardiol 1968; 21:413-419.[CrossRef][Medline]
- Burke A, Virmani R. Tumors of the heart and great vessels: Atlas of tumor pathology Fasc 16, ser 3. Washington, DC: Armed Forces Institute of Pathology, 1996.
- Vander Salm TJ. Unusual primary tumors of the heart. Semin Thorac Cardiovasc Surg 2000; 12:89-100.[Medline]
- Janigan DT, Husain A, Robinson NA. Cardiac angiosarcomas: a review and a case report. Cancer 1986; 57:852-859.[CrossRef][Medline]
- Raaf HN, Raaf JH. Sarcomas related to the heart and vasculature. Semin Surg Oncol 1994; 10:374-382.[Medline]
- Butany J, Yu W. Cardiac angiosarcoma: two cases and a review of the literature. Can J Cardiol 2000; 16:197-205.[Medline]
- Mader MT, Poulton TB, White RD. Malignant tumors of the heart and great vessels: MR imaging appearance. RadioGraphics 1997; 17:145-153.[Abstract]
- Bruna J, Lockwood M. Primary heart angiosarcoma detected by computed tomography and magnetic resonance imaging. Eur Radiol 1998; 8:66-68.[CrossRef][Medline]
- Marofioti T, Castorino F, Gula G. Cardiac angiosarcoma: histological, immunohistochemical and ultrastructural study. Pathologica 1993; 85:103-111.[Medline]
- Uberfuhr P, Meisner B, Fuchs A, et al. Heart transplantation: an approach to treating primary cardiac sarcoma? J Heart Lung Transplant 2002; 21:1135-1139.[CrossRef][Medline]
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History
Imaging Findings
