Malignant jugulotympanic paraganglioma.

Abstract

Jugulotympanic paragangliomas are neoplasms that arise in the jugular bulb (glomus jugulare tumor) or temporal bone (glomus tympanicum tumor). They are histologically similar to carotid body tumors; both are derived from extra-adrenal paraganglia. Of only 20 metastases reported, five had biopsy-proved bone involvement. We present a review of the literature of metastatic jugulotympanic paragangliomas and report a case that recurred locally with regional metastases 10 years after initial presentation and metastasized to spine 13 years later. Histologic data are reviewed, and potential treatment regimens are contrasted.