Metachronous bilateral nonfunctional intercarotid paraganglioma (carotid body tumor) and functional retroperitoneal paraganglioma: report of a case and review of the literature.

BACKGROUND: The occurrence of an intercarotid paraganglioma and a paraganglioma in an anatomic distribution away from the head and neck in the same patient is rare. We have treated a patient with this combination of tumors and report this unusual case with the purpose of reviewing the paraganglion system. We endeavor to clarify the confusion that exists by use of the terms chemodectoma, glomus tumors, nonchromaffin paragangliomas, and pheochromocytoma and to present a rational nomenclature based on their ontogeny. METHODS: A computer-generated list of articles on this subject was obtained, and all articles relative to this rare combination of tumors were reviewed. RESULTS: We found 31 other cases. Functional intercarotid paragangliomas are extremely rare, with only five cases having been reported. CONCLUSIONS: All hypertensive patients who are found to have an intercarotid paraganglioma should be investigated for a functional tumor. Family members should also be made aware of their probabilities of having these tumors because a familial trend has been documented in certain cases.