An intrajugular paraganglioma. Unusual presentation of a classical tumor

Paragangliomas arise from the extraadrenal neuroendocrine system. They are locally aggressive tumors, causing adjacent invasion, bone destruction and compression related symptoms. We present a 35-years-old woman with a peculiar paraganglioma lacking all these features, and strictly located within the jugular vein. Differential diagnosis is detailed since other entities could have dissimilar clinical behavior. To the best of our knowledge, this is a very unusual site of occurrence for paragangliomas, and only two other comparable cases have been described.