Abstract
Angiosarcoma is the most invasive and malignant cardiac tumor and most commonly originates from the right atrium. Early diagnosis is essential, and echocardiography has an important role in diagnosis. This tumor grows aggressively, and metastases to other sites makes it difficult to control. Surgical treatment remains the best option for patients who do not respond to chemoradiotherapy. We herein report a case of a 17-year-old patient with cardiac angiosarcoma who presented with dyspnea, chest pain, dry cough, and fever. Although we considered the most probable diagnosis to be constrictive pericarditis, pathologic examination revealed a primary angiosarcoma originating from the pericardium. The patient underwent total pericardiectomy. However, despite receiving chemotherapy for 2 weeks postoperatively, she developed complications including leukopenia and eventually died of respiratory failure. Late diagnosis of angiosarcoma often occurs, resulting in progression to end-stage disease and a very poor prognosis. Therefore, a thorough understanding of this entity, knowledge of its pitfalls in management, and establishment of an accurate treatment guideline would help to develop a reliable and life-saving treatment approach for these patients.