Gangliocytoma: outcome of a rare silent pituitary tumour

Abstract

The most common finding in front of a pituitary incidentaloma is a silent pituitary adenoma. We describe a 59 years old woman with a pituitary gangliocytoma and her follow-up after 1 year. Hormonal exploration only evidenced partial corticotropic insufficiency. A trans-sphenoidal surgery was performed due to the tumour's suprasellar expansion. Gangliocytoma is a benign tumour of unknown prevalence, belonging to central nervous system tumour with neuronal differentiation, and 129 cases have been reported in the literature. growth hormone (GH), adrenocorticotropic hormone (ACTH) or prolactin secretions have been reported, as these ganglion cell-like mature neurons are usually mixed with secreting pituitary endocrine cells. We report a case with a pure gangliocytoma devoid of symptomatic endocrine secretion, not surrounded by pituitary endocrine tumour cells. Immunochemistry of the tumour was positive for hypothalamic growth hormone releasing hormone (GHRH) and pituitary hormones, such as GH and ACTH. Hence, this immunoexpression was not associated with peripheral hormonal secretions, suggesting biologically inactive hypothalamopituitary hormones.

Mixed somatotroph adenoma-gangliocytoma: A rare sellar combined tumor

“…These pituitary tumors have also been called pituitary adenoma-neuronal choristoma (PANCH), pituitary adenoma with neuronal choristoma, and pituitary adenoma with gangliocytic component. The pituitary gangliocytoma is a rare brain tumor, usually associated with an adenoma leading to hypersecretion of pituitary hormones, with an approximate incidence of 149 cases in the literature according to our review [1,[7][8][9][10][11][12][13]. Pituitary gangliocytomas consist of ganglion cells with a variably dense fibrillary substrate, frequently associated with the adenomatous proliferation of pituitary cells [14].…”

“…There are 130 cases of gangliocytomas associated pituitary adenomas reported in the review of Cossu et al in 2016 [1], and there are more than 19 cases reported afterward [7][8][9][10][11][12][13]. The incidence of this association varies from 0.52% to 1.26% in different series [14,15].…”

Pituitary Gangliocytoma Producing TSH and TRH: A Review of “Gangliocytomas of the Sellar Region”

“…Additionally, among these 10 recent cases, 4 (40%) demonstrated immunoreactivity to pituitary hormones. In some cases, the tumor cells exhibited overlapping immunoreactivity to the same hypothalamic–pituitary hormonal axis ( 35 , 37 ). Most of these pathological findings correspond to the endocrinological symptoms of the respective cases.…”