Late-Onset Familial Primary Cardiac Angiosarcoma in Two Sisters

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 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.

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.