Primary cardiac undifferentiated sarcoma of the right atrium presenting with cardiac tamponade

Abstract

This report illustrates the serial imaging of a primary cardiac undifferentiated sarcoma of the right atrium using echocardiography, chest X-ray, and computed tomography. Transthoracic echocardiography on presentation showed an extensive mass of the right atrial free wall with an impending cardiac tamponade. Symptoms were controlled with pericardiocentesis, pericardial window, and radiotherapy but recurred 8 months later with pleural effusion and tumor spread to the great arteries. Primary cardiac sarcoma (PSC) is a rare and aggressive malignancy that is usually diagnosed late due to its nonspecific symptoms. Cytology and cardiac biopsy may be negative, and suspicion for the tumor is warranted in recurrent pericardial effusion. Analogous to parietal pleural biopsy in lung tumors with pleural effusion, parietal pericardial biopsy may be positive in PSC of the right atrium with pericardial effusion. Echocardiography is the major diagnostic tool and aids pericardiocentesis. Pericardial window may be useful for recurrent pericardial effusion but does not preclude its reaccumulation. There is no proven effective treatment for PSC, and treatments include surgical resection, cardiac transplant, chemotherapy, and radiotherapy. Despite its poor prognosis, symptomatic relief is important and attainable.