Unusual survival time of primary cardiac sarcoma of the right ventricle.

Primary cardiac sarcomas are rare tumors with unfavorable prognosis. We report a 69-year-old male with a right ventricular mass diagnosed as primary malignant cardiac sarcoma with unexpected long survival of 16.5 months.

INTRODUCTION

Primary cardiac sarcomas are rare malignant tumors with poor prognosis for survival ranging from 6 to 12 months. The prevalence of primary cardiac sarcoma in autopsy series is 0.001–0.28%.[1] In one of the series, the incidence was reported to range from 0.0017 to 0.019%.[23] The usual sites are the atrium, ventricle, pericardium or great vessels.[4] The therapeutic options include surgery, chemotherapy and radiotherapy alone or in combination.[5]

CASE PRESENTATION

A 69-year-old male with diabetes mellitus presented with epigastric pain and weight loss for 3 months. Examination revealed low grade fever and markedly raised jugular venous pressure. Chest radiograph demonstrated cardiomegaly and small bilateral pleural effusions. Transthoracic echocardiography demonstrated large pericardial effusion with cardiac tamponade. He underwent emergency pericardiocentesis and 1060 ml of hemorrhagic fluid was aspirated.

Percutaneous pericardial biopsy was done to look for specific etiology. After pericardial biopsy, a 4-F pigtail catheter was left in the pericardial cavity for further aspiration. Exactly 5600 ml of hemorrhagic pericardial fluid was aspirated during the following 2 weeks. Pericardial fluid Gram stain and culture were negative for pyogenic or tuberculosis infection. Cytology did not reveal any malignant etiology. Repeat echocardiographic imaging revealed a large solid mass (7 × 10 × 8 cm) involving the right ventricular inflow, the body of the right ventricle and right ventricular outflow tract postero-medially. The mass appeared relatively fixed with possible myocardial infiltration [Figure 1a–c]. Cardiac catheterization and hemodynamic assessment showed biventricular heart failure. Right ventricular angiogram demonstrated a filling defect in the postero-inferior aspect of the right ventricle. Biopsy taken from that area using a bioptome revealed primary cardiac sarcoma of the malignant fibrous histiocytoma sub-group. He was offered tumor resection with chemotherapy but he did not accept. Initial computed tomography (CT) scan did not reveal any extracardiac tumor or metastasis. Echocardiographic evaluation at 6 and 8 months interval revealed enlarging tumor mass. The last echocardiogram demonstrated a huge right ventricular tumor measuring 20 × 10 cm, filling the entire right ventricular cavity and infiltrating through the right ventricular wall into the liver [Figure 2a and b]. He also had small pericardial effusion, bilateral pleural effusions, ascites as well as hepatic and splenic metastasis. He continued to refuse any treatment and expired at home 16.5 months after the initial presentation.

Figure 1a

2-Dimensional echocardiographic imaging (apical 4 chamber view) demonstrating dilated right side of the heart with most of the right ventricular cavity occupied by the tumor mass

Figure 1c

2D echocardiogram parasternal short axis view showing the tumor mass at the right ventricular outflow and inflow

Figure 2a

Subcostal echocardiographic view demonstrating the tumor mass extending outside the right ventricle infiltrating into the liver

Figure 2b

Subcostal echocardiographic view showing spleen an echogenicity suggesting splenic infiltration

2D apical 4 chamber view showing forward tricuspid flow through the residual RV cavity

DISCUSSION

Cardiac sarcomas are usually asymptomatic until advanced, or present after variable periods of symptoms that are often nonspecific ranging from a few weeks to several months.[6] They manifest by pericardial effusion with tamponade, local invasion causing arrhythmias, obstruction to blood flow and interference with valve function, embolic phenomena, or systemic symptoms of dyspnea, fever, malaise and weight loss. In one of the published series, 29% of patients had pericardial effusion at presentation.[3]

Our patient had massive pericardial effusion with tamponade at the initial presentation. He underwent emergency pericardiocentesis and the repeat echocardiography could demonstrate the tumor mass in the right ventricle. Dyspnea is the commonest symptom in 60%, followed by chest pain in 28%, congestive heart failure in 28%, palpitations in 24%, fever in 14%, myalgia in 10% and embolism in 5%.[1] Although in adults cardiac tumors are mostly benign, 25% can be malignant and one-third of these are angiosarcomas.[7-9] Cardiac sarcoma is a rapidly growing tumor with local invasion and systemic metastases which result in dismal prognosis. Therefore, early recognition and characterization of their pathoanatomy is crucial. In previous studies, metastases were present in 66–89% of patients at the time of diagnosis.[10-12] Burke et al,[13] reported significantly improved survival period of 12 months for patients with primary cardiac angiosarcoma who received treatment, while for the untreated, the median survival was only 3 months. Mean survival for most sarcomas is about 9–11 months. Median survival ranges from 24 months after resection versus 10 months with either incomplete or no resection.[2] This case report highlights an unusual clinical course of survival for 16.5 months despite being without treatment, leading to local infiltration and distant metastasis.

CONCLUSION

This is a case of infiltrating cardiac sarcoma with unexpected survival for 16.5 months without definitive therapy. Although the patient presented with nonspecific symptoms and cardiac tamponade, echocardiography was extremely useful to diagnose the cardiac sarcoma and to document its progress.