'Gangliocytomas' of the pituitary: a heterogeneous group of lesions with differing histogenesis

Abstract

Hamartomatous or neoplastic ganglion cells in the sella turcica are an unusual cause of symptoms. They have been reported in association with a functioning or nonfunctioning pituitary adenoma, with pituitary cell hyperplasia, and occasionally as masses unassociated with an adenoma, again with variable endocrinologic findings. Fewer than 50 cases of intrasellar ganglion cell lesions have been reported in the literature, only six of them associated with Cushing's syndrome. We describe the clinicopathologic features of another eight patients, three of whom presented with acromegaly, four with apparently nonfunctioning adenohypophyseal masses, and one with Cushing's syndrome. On histology, six of them were found to have sparsely granulated growth hormone (GH)-producing adenomas with ganglion cell areas, one appeared to have a gangliocytoma not associated with an adenoma, whereas the eighth had a ganglion cell lesion in the posterior pituitary. The morphologic and immunohistochemical findings suggest that the ganglion cell component of seven of these tumors has resulted from neuronal differentiation in a GH-producing adenoma, despite the lack of demonstrable adenoma in one case. A true sellar "gangliocytoma" or hamartoma of ectopic hypothalamic-type neurons appears to be a rarer explanation for the presence of ganglion cells in a pituitary biopsy.