Pericardial sarcomas are extremely rare aggressive neoplasms. Non-specific symptoms and incidental discovery are usually the rule. Multimodality imaging is extremely important for diagnosis and tissue characterisation of all cardiac masses. Despite treatment, pericardial sarcomas are considered extremely fatal. We encountered a 27-year-old female patient who presented to our facility with progressive dyspnoea. On examination, clinical signs of cardiac tamponade were appreciated, transthoracic echocardiography revealed a tamponading pericardial effusion and a large heterogeneous pericardial mass. Pericardiocentesis revealed haemorrhagic fluid. Subsequently, CT revealed a pericardial mass compressing the right atrium. Excision biopsy showed a well-circumscribed mass, and cut sections showed friable grey–white tissue with areas of haemorrhage and necrosis. Pathological examination confirmed the diagnosis of high-grade undifferentiated sarcoma of the pericardium. The patient was started on adjuvant chemotherapy and radiotherapy. Follow-up after 1 year showed no relapse.
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