Late-onset familial primary cardiac angiosarcoma in two sisters

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ID: 321803
2026
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Ranked #12 of 79 articles by views in Interdisciplinary CardioVascular and Thoracic Surgery

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Abstract
Primary cardiac angiosarcoma is an exceptionally rare malignancy. Familial occurrences are even less common and associated with hereditary cancer syndromes; notably, all previously reported familial cases occurred in young individuals. We encountered two elderly sisters who both developed morphologically similar primary cardiac angiosarcomas arising from the right atrium. The elder sister presented with nocturnal wheezing, and a large right atrial tumor was found at 70. She underwent surgical resection followed by radiotherapy. The younger sister presented with a similar cough, and screening transthoracic echocardiography revealed a right atrial mass at 74. She received similar treatment. These cases suggest a potential hereditary predisposition and further highlight that familial occurrence of primary cardiac angiosarcoma can manifest even at advanced age, challenging conventional understanding of this disease.
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openalex_W7169878835 Use this key to autocite in the manuscript while using SciMatic Manuscript Manager or Thesis Manager
Authors Hiroto Kawakami, Hiroshi Tsuneyoshi, Shoichi Kyo, Makoto Suzuki
Journal Interdisciplinary CardioVascular and Thoracic Surgery
Year 2026
DOI
10.1093/icvts/ivag114
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