Secreting jugulotympanic paraganglioma with venous involvement into the thorax.

Abstract

Paragangliomas are highly vascular tumors that arise from chief cells in extra-adrenal paraganglia of the autonomic nervous system. Jugulotympanic paragangliomas involve the middle ear and jugular fossa. Secreting jugulotympanic paragangliomas with thoracic extension are rare. We report a case with unexpected involvement of the internal jugular vein. Otolaryngologists should be aware that the jugulotympanic paragangliomas traverse variable anatomic pathways. Multiple imaging options, including magnetic resonance venography, are useful and should include the neck for definitive characterization of tumor anatomy. Patients with refractory hypertension and masses suspicious for paraganglioma should be examined for functional tumors.