Late-onset familial primary cardiac angiosarcoma in two sisters

Scritto il 21/07/2026
da Hiroto Kawakami

Interdiscip Cardiovasc Thorac Surg. 2026 Jul 21:ivag114. doi: 10.1093/icvts/ivag114. Online ahead of print.

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.

PMID:42479150 | DOI:10.1093/icvts/ivag114