Asian Cardiovasc Thorac Ann 2008;16:154-156
© 2008 Asia Publishing EXchange Ltd
Angiosarcoma Presenting as Syncope
Sushma Nayar, MD,
Pradeep G Nayar, DNB1,
KM Cherian, FRACS2
Department of Pathology
1 Department of Cardiology
2 Department of Cardiothoracic Surgery, International Center for Cardio Thoracic and Vascular Diseases, Dr KM Cherian Heart Foundation Chennai, India
For reprint information contact: Sushma Nayar, MD, Tel: 91 44 2656 7200, Fax: 91 04 2656 5150, Email: divya_s35{at}hotmail.com, International Center for Cardio Thoracic and Vascular Diseases (A Unit of Frontier Lifeline), Dr KM Cherian Heart Foundation, R 30 C Ambattur Industrial Estate Road, Mogappair, Chennai 600 101, India.
 |
ABSTRACT
|
|---|
A 31-year-old lady presented with anemia and syncope. Echocardiography revealed massive pericardial effusion with a right atrial mass. Transesophageal echocardiography, computed tomography and magnetic resonance imaging scans confirmed presence of a right atrial mass. Histopathology revealed a high grade angiosarcoma. Complete resection was done and the patient was referred to an oncology unit for further management. After three months the patient had extensive metastasis and succumbed to the disease. This case report highlights the clinical presentation, rapid and aggressive course of cardiac angiosarcomas.
|
INTRODUCTION
|
|---|
Primary malignant tumors of the heart are rare. On account of non-specific symptoms the disease is usually diagnosed only after regional spread has occurred. Our patient a 31-year-old lady, presented with anemia and syncope. Echocardiography, CT and MRI delineated the tumor in the right atrium before regional spread. Histopathology showed a high grade angiosarcoma. Though complete removal of the tumor is the main stay of the treatment, it is difficult due to its location. Rapid dissemination occurs due to the aggressive nature of the disease.
|
CASE REPORT
|
|---|
A 31-year-old previously healthy lady presented with a history of one episode of syncope one month previously. On echocardiography, massive pericardial effusion with a right atrial mass attached to the lateral wall was seen (Figure 1
).1 A quantity of 1000 mL of sero-sanguinous fluid was tapped and sent for biochemical, microbiological and cytological studies. The fluid was found to be an exudate, negative for acid-fast bacilli and malignant cells.1
View larger version (43K):
[in this window]
[in a new window]
|
Figure 1. Transthoracic echocardiography image showing the RA mass (arrow). LA= left atrium, LV= left ventricle, RA= right atrium, RV= right ventricle.
|
|
On repeat echocardiography and transesophageal echocardiography (TEE), the mass appeared very vascular and did not show any attachment to the inter-atrial septum.1 The mass was seen extending to the lumen of the right atrium. With a suspicion of malignancy, computed tomography (CT) and magnetic resonance imaging (MRI) was done which confirmed the presence of a soft tissue mass with increased vascularity in the right atrium. The lung fields were clear. There was no evidence of extra-cardiac origin of the tumor or evidence of lymph node involvement.2 The patient also had co-incident anemia (Hb 8.2 g·dL–1). Further investigation revealed iron-deficiency anemia. Two units of packed cells followed by hematinics were given. Preoperatively, the hemoglobin was 11.0 g·dL–1.
The patient was taken up for surgical removal of the mass. On sternotomy, the pericardium was thickened. There were a lot of tortuous vessels over the pericardium. Adhesions were present over the pericardium. The right atrial wall was friable and adherent to the pericardium. The tumor mass involved the lateral wall of the right atrium, infiltrating the wall and growing into the right atrial cavity. The inter-atrial septum, tricuspid valve, superior vena cava and inferior vena cava openings were free of disease. The tumor was friable.1,2 The right atrial mass was removed and sent for histopathology, and re-construction of the right-atrial wall using bovine pericardium was performed. The postoperative period was uneventful.
Histopathology showed the tumor consisting of spindle-shaped cells lining vascular spaces. Numerous mitotic figures were seen. Areas of hemorrhage and necrosis were also seen. Tufting of the lining endothelial cells was present (Figure 2). The tumor was infiltrating the right atrial myocardium and was 0.1 cm from the closest epicardial margin. A provisional diagnosis of angiosarcoma was made.
Immunohistochemistry confirmed the vascular nature of the tumor (CD31 positive, CD34 focally positive, desmin negative, CD99 negative and c-kit negative). A diagnosis of high grade angiosarcoma was made. The patient was sent to an oncology center for further management. On review at three months, repeat CT scan showed multiple secondaries in the lung and liver. Subsequently she succumbed to the disease.
|
DISCUSSION
|
|---|
Primary malignant tumors of the heart are rare, angiosarcoma being the most common. The right atrium is the most common site of the tumor.1,2,3 Prognosis is typically poor and is usually less than 1 year. Aggressive behavior and delayed diagnosis on account of non-specific symptoms results in the disease being diagnosed usually only after regional spread has occurred, unlike in our case. Farah et al have reported a similar case presenting with pericardial tamponade and syncope.1 The lateral wall of the right atrium is the most common site, the septum being spared in most cases.3,4 Our case was consistent with the classical description. Clinical features depend on the size and location of the tumor. The atrial location may present as pericardial effusion, inflow obstructions, SVC and IVC obstruction, systemic or pulmonary emboli,2 supra-ventricular arrhythmias, or as conduction abnormalities such as low-voltage complexes2 from pericardial infiltration (as in our case). A close relationship between the EKG lead changes and the anatomic location of the tumor has been seen. Patients thus present with signs of right heart failure or pericardial effusion.1,3,4
Any mass arising from the lateral wall of the atrium with lack of attachment to the inter-atrial septum should raise a suspicion of neoplasia.5 Investigation by TEE is preferred to delineate the origin, size and extension of the tumor. Computed tomography and MRI give additional information regarding spread of the disease. Coronary angiograms with cardiac catheterization help in optimum evaluation of peri-tumoral neo-vascularization and extent of great vessel involvement if any, though it was not performed in our case.
Complete removal of the tumor is the mainstay of treatment.5 Postoperative mediastinal radiation has been seen to prolong survival especially in cases of metastasis. Cardiac transplantation has been tried in selected cases with localized disease. However, whatever the mode of therapy used, the outlook remains bleak. Few cases of angiosarcoma respond to Gleevec® (Imatinib Mesylate, ST1571). However, this tumor being c-kit negative may not have benefited from this mode of treatment.
|
CONCLUSION
|
|---|
Angiosarcoma presenting as syncope has a rapid and aggressive course; complete surgical resection is the mainstay of treatment.
|
ACKNOWLEDGMENTS
|
|---|
We thank Dr. P Mallikarjun (Apollo Speciality Hospital) for performing immunohistochemistry, Mrs. Meena Rani, echocardiographer for the echo pictures and Ms. Revathy Vijayakumar for secretarial assistance.
|
REFERENCES
|
|---|
- Farah HH, Jacob M, Aragam J. Images in cardiology: A case of cardiac angiosarcoma presenting as pericardial tamponade. Heart 2001;86:665.[Free Full Text]
- Dennig K, Lehmann G, Richter T. An angiosarcoma in the left atrium. N Engl J Med 2000;342:443–4.[Free Full Text]
- Putnam JB Jr, Sweeney MS, Colon R, Lanza LA, Frazier OH, Cooley DA. Primary cardiac sarcomas. Ann Thorac Surg 1991;51:906–10.[Abstract]
- Ananthasubramaniam K, Farha A. Primary right atrial angiosarcoma mimicking acute pericarditis, pulmonary embolism, and tricuspid stenosis. Heart 1999;81:556–8.[Abstract/Free Full Text]
- Burke AP, Cowan D, Virmani R. Primary sarcomas of the heart. Cancer 1992;69:387–95.[Medline]
TOP
ABSTRACT
INTRODUCTION
