Familial occurrence of paragangliomas

The occurrence of paragangliomas in three members of the same family is reported. It concerned a left carotid body tumor in one patient, a left carotid body tumor in her older sister, and a concomitant jugulotympanic and vagal body tumor in their younger brother. The radiological diagnosis in these patients was based on the typical hypervascular character in specific locations both on angiography and computed tomography. The familial occurrence of these lesions should be kept in mind whenever a chemodectoma is found, even in the absence of clinical symptoms in the direct relatives of the patients.