Abstract Background Angiosarcoma is a malignant rare tumor that originates from vascular endothelial cells that cover lymphatic or blood vessels. Cardiac angiosarcoma is the most prevalent sarcoma entail the heart. It has low incidence rate and poor prognosis. Our effort through this report was raising awareness of uncommon manifestations of this disease and showing the importance of appropriate diagnosis and treatment. Case presentation We present a case of cardiac angiosarcoma in a young female whose symptoms included dyspnea and hemoptysis with a history of pericardial effusion and a past history of cardiac surgery for suspected atrial Myxoma. She had history of several hospitalizations and relapse of symptoms a few months after each hospital discharge. Conclusions The unspecific symptoms of cardiac angiosarcoma made it difficult to make in time diagnose and appropriate treatment. Awareness of unspecific presentations of cardiac angiosarcoma is necessary for proper diagnosis and treatment while delayed diagnosis may worsen the prognosis and even lead to death.
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