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Primary cardiac tumors are a rare disease, with 20% of the cases being malignant. Among them, angiosarcoma is characterized by a short clinical course and poor prognosis, even after surgery, chemotherapy, and radiation therapy. We present a 67-year-old woman diagnosed with a primary malignant tumor (angiosarcoma) infiltrating the right atrial myocardium.

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Angiosarcomas are rare and often a fatal manifestation of cardiac tumors. This paper presents a case of a sarcoma with protrusion through the mitral valve. Angiographic findings demonstrated a tumor blush, supplied by the right coronary and left anterior descending arteries.

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A 75-year-old patient with a history of mitral and aortic valve replacement surgery 7 years ago, presented with progressive dyspnea. Transesophageal echocardiogram showed a mass suggestive of bioprosthetic mitral valve thrombosis. We present the investigation process using imaging, surgical findings, nuclear medicine, and histopathology that result in the diagnosis of cardiac angiosarcoma.

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Purpose: Cardiac angiosarcoma (CAS) is a rare, aggressive malignancy with limited treatment options. Both sporadic and familial cases occur, with recent links to germline POT1 mutations. The genomic landscape of this disease is poorly understood.

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