{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,24]],"date-time":"2026-07-24T21:10:27Z","timestamp":1784927427478,"version":"3.55.0"},"reference-count":5,"publisher":"Oxford University Press (OUP)","issue":"7","license":[{"start":{"date-parts":[[2026,7,21]],"date-time":"2026-07-21T00:00:00Z","timestamp":1784592000000},"content-version":"vor","delay-in-days":20,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,7,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Introduction: 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.<\/jats:p>\n                  <jats:p>Case: We encountered 2 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 tumour was found at age 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 age 74. She received similar treatment.<\/jats:p>\n                  <jats:p>Discussion: These cases suggest a potential hereditary predisposition and further highlight that familial occurrence of primary cardiac angiosarcoma can manifest even at advanced age, challenging the conventional understanding of this disease.<\/jats:p>","DOI":"10.1093\/icvts\/ivag114","type":"journal-article","created":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T11:22:03Z","timestamp":1784546523000},"source":"Crossref","is-referenced-by-count":0,"title":["Late-Onset Familial Primary Cardiac Angiosarcoma in Two Sisters"],"prefix":"10.1093","volume":"41","author":[{"ORCID":"https:\/\/orcid.org\/0009-0009-6433-0934","authenticated-orcid":false,"given":"Hiroto","family":"Kawakami","sequence":"first","affiliation":[{"name":"Department of Cardiovascular Surgery, Shizuoka General Hospital , Shizuoka 420-8527,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroshi","family":"Tsuneyoshi","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Surgery, Shizuoka General Hospital , Shizuoka 420-8527,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shoichi","family":"Kyo","sequence":"additional","affiliation":[{"name":"Department of Cardiovascular Surgery, Shizuoka General Hospital , Shizuoka 420-8527,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Makoto","family":"Suzuki","sequence":"additional","affiliation":[{"name":"Department of Pathology, Shizuoka General Hospital , Shizuoka 420-8527,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2026,7,21]]},"reference":[{"key":"2026072416561373100_ivag114-B1","doi-asserted-by":"publisher","first-page":"103","DOI":"10.12659\/MSM.889875","article-title":"Primary cardiac angiosarcoma - a review","volume":"20","author":"Patel","year":"2014","journal-title":"Med Sci Monit"},{"key":"2026072416561373100_ivag114-B2","doi-asserted-by":"crossref","first-page":"1576","DOI":"10.1016\/j.athoracsur.2006.03.006","article-title":"Familial cardiac angiosarcoma","volume":"82","author":"Keeling","year":"2006","journal-title":"Ann Thorac Surg"},{"key":"2026072416561373100_ivag114-B3","doi-asserted-by":"crossref","first-page":"392","DOI":"10.1067\/mtc.2002.122314","article-title":"Familial angiosarcoma of the heart","volume":"124","author":"Casha","year":"2002","journal-title":"J Thorac Cardiovasc Surg"},{"key":"2026072416561373100_ivag114-B4","doi-asserted-by":"crossref","first-page":"8383","DOI":"10.1038\/ncomms9383","article-title":"A mutation in the POT1 gene is responsible for cardiac angiosarcoma in TP53-negative Li-Fraumeni-like families","volume":"6","author":"Calvete","year":"2015","journal-title":"Nat Commun"},{"key":"2026072416561373100_ivag114-B5","doi-asserted-by":"crossref","first-page":"1091","DOI":"10.1158\/1078-0432.CCR-24-3277","article-title":"Genomic profiling of cardiac angiosarcoma reveals novel targetable KDR variants, recurrent MED12 mutations, and a high burden of germline POT1 alterations","volume":"31","author":"Odintsov","year":"2025","journal-title":"Clin Cancer Res"}],"container-title":["Interdisciplinary CardioVascular and Thoracic Surgery"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/icvts\/advance-article-pdf\/doi\/10.1093\/icvts\/ivag114\/69696604\/ivag114.pdf","content-type":"application\/pdf","content-version":"am","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/icvts\/article-pdf\/41\/7\/ivag114\/69696604\/ivag114.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/icvts\/article-pdf\/41\/7\/ivag114\/69696604\/ivag114.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,24]],"date-time":"2026-07-24T20:56:24Z","timestamp":1784926584000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/icvts\/article\/doi\/10.1093\/icvts\/ivag114\/8739199"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,7,1]]},"references-count":5,"journal-issue":{"issue":"7","published-print":{"date-parts":[[2026,7,1]]}},"URL":"https:\/\/doi.org\/10.1093\/icvts\/ivag114","relation":{},"ISSN":["2753-670X"],"issn-type":[{"value":"2753-670X","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2026,7]]},"published":{"date-parts":[[2026,7,1]]},"article-number":"ivag114"}}