Primary cardiac angiosarcoma is a rare, aggressive tumor that can present with recurrent pericardial effusion and cardiac tamponade, requiring histopathology for definitive diagnosis.
Case of cardiac angiosarcoma at age 30 extends known younger presentations; leaves open need for prospective data on early detection and management.
Primary cardiac angiosarcoma is a rare and aggressive malignant mesenchymal neoplasm. They show endothelial differentiation in morphology and immunohistochemistry. There is a male predominance, common in the western world, and the age of presentation being fourth to sixth decades. The prognosis is worse as compared to other sarcomas due to the high chance of developing cardiac tamponade. We report the case of a 30-year-old male who presented with chest pain and shortness of breath. The patient complained of pain due to recurrent pericardial effusion which was drained and sent for cytological examination. The fluid was negative for any malignancy and inflammatory pathology. However, his symptoms worsened and a cardiac magnetic resonance imaging was suggestive of a right atrial tear and pseudoaneurysm suspected iatrogenic injury from the previous pericardiocentesis at another hospital before admission. The patient underwent emergency sternotomy for cardiac tamponade with right atrial repair and the removed clot and pericardium were sent for histopathology. Histopathology and immunohistochemistry surprisingly revealed angiosarcoma in the pericardial patch tissue. Unfortunately, the patient died postoperatively. Angiosarcomas are the most common cardiac malignant tumors. It has an aggressive course with poor prognosis and variable clinical presentation. Histopathology with immunohistochemistry plays a definite role in diagnosing these tumors. Hence, its awareness is a must among clinicians and pathologists.
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Saraf et al. (2021) studied this question.
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