Intrasellar paraganglioma: report of a case in a sibship of von Hippel-Lindau disease.

Although uncommon, paragangliomas are known to affect the central nervous system. Those that arise in the sellar region are particularly rare. We report the clinicopathological features of a paraganglioma occurring in the setting of von Hippel-Lindau disease, a disorder in which paragangliomas are known to occur. We also present a review of the literature.