Malignant jugulotympanic paraganglioma

Jugulotympanic paragangliomas are neoplasms that arise in the jugular bulb (glomus jugular 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.