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Journal of Radiological Review 2020 November-December;7(6):456-67

DOI: 10.23736/S2723-9284.20.00071-3


language: English, Italian

Description of a rare case of right atrial angiosarcoma

Angela TETI 1 , Valentina SETTINO 2, Domenico CONSOLE 2, Valentina CERRA 2, Anna ROTUNDO 2, Domenico LAGANÀ 2

1 Unit of Radiology, Magna Grecia University, Catanzaro, Italy; 2 Division of Radiology, Department of Clinical and Experimental Medicine, Mater Domini University Hospital, Catanzaro, Italy


Primary cardiac tumor is a rare disease with an autoptic incidence of 0.001-0.03%. About a quarter of primary cardiac tumors show some malignancy aspects and 95% of these are sarcomas. Clinical onset that characterizes patients with primary heart cancer is varied, it often simulates cardio-pulmonary pathologies and the most frequent presentation is heart failure. Dyspnea is the most common symptom in patients with primary cardiac sarcoma. In about 80% of cases, metastatic lesions are already present at the time of diagnosis. The most affected organs are lungs but also lymph nodes, bone, liver, brain, intestine, spleen, adrenals, pleura, diaphragm, kidneys, thyroid and skin could be affected. Chemotherapy and radiation therapy have not proven effective. Aggressive surgical therapy is palliative on symptoms and improves survival that is about 11 months on average. Below it is reported the case of a young woman, in apparent state of good health, who accessed to the Emergency Room due to a major lipothymic event with consequent finding of conspicuous pericardial effusion and a lesion in the right atrium that turned out to be a poorly differentiated cardiac angiosarcoma. The case is of particular interest because it presents some peculiar aspects in terms of clinical presentation, biological behavior of the tumor, late appearance and localization of metastatic lesions and survival. It also underlines how multimodal imaging plays a fundamental role in the diagnosis and choice of therapeutic strategy.

KEY WORDS: Sarcoma; Heart neoplasms; Multimodal imaging

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