Mixed Pituitary Adenoma and Gangliocytoma Associated with A cromegaly

Abstract

A gangliocytoma in the sellar region is very rare. We report a case of an intrasellar gangliocytoma com plicated by pituitary adenoma presenting with acromegaly. A 52-year-old female was admitted to our hospital with headache, mild acromegaly, and bitemporal hemianopsia, and endocrinological study found a high serum level of growth hormone (GH). A computed tomographic scan revealed a tumor in the sellar region, which was almost totally removed by trans-sphenoidal surgery . Histo logical examination of the resected specimen showed diffuse, chromophobe-type pituitary adenoma , partially containing cholesterin clefts. Areas of clusters of dysmorphic neurons, adjacent to or mixed with pituitary adenoma, were diagnosed as gangliocytoma.The immunohistochemical exami nation showed GH-releasing hormone (GRH)-positive dysmorphic neurons and GH-positive pituitary adenoma. We consider that the trophic effect of GRH secreted by the neurons of GRH-producing in trasellar gangliocytoma probably caused the GH-producing pituitary adenoma.

Ganglion cell tumours in the sella turcica in close morphological connection with pituitary adenomas

“…They are composed of mature ganglion cells with or without a glial component. Gangliocytomas of the pituitary gland are exceptionally rare and usually coexist with a pituitary adenoma, most commonly a GH-secreting adenoma, with the clinical manifestation of acromegaly [4][5][6]12,17,23,27,32,35,39,44,46]; less often, they are associated with ACTH-producing adenomas, and present with Cushing's disease [25,36,40].…”

“…Such neoplasms occur without evidence of adjacent adenoma only occasionally. The majority of ganglion cell tumours appear in association with growth hormone-secreting pituitary adenoma presenting with acromegaly [2,[4][5][6]12,[17][18][19]23,25,32,38,40,42]. They appear less often with adrenocorticotropic hormone-producing adenomas with clinical presentation of Cushing's disease [25,36,40].…”

Mixed gangliocytoma-pituitary adenoma containing GH and GHRH co-secreting adenoma cells

“…On the other hand, both components of mixed gangliocytoma-pituitary adenoma were demonstrated to produce the hypothalamic releasing hormones (3). All previous reports of mixed gangliocytoma-pituitary adenoma investigating GHRH immunoreactivity have implied GHRH production in the gangliocytic components (Table 1) (1, 5, 6, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19). However, our present case demonstrated that only the adenomatous components may produce GHRH in GH-secreting mixed gangliocytoma-pituitary adenoma.…”

An unusual association of a sellar gangliocytoma with a prolactinoma

“…Approximately 55 cases of sellar gangliocytomas have been reported in the literature only five of them associated with PRL hypersecretion [1][2][3][4]. GH hypersecretion was present in the majority of patients with hormonal excess.…”

“…They may occur in association with functioning, usually GH-secreting-, or non-functioning pituitary adenomas. The two cell types, adenomatous and neuronal, may be admixed or may coexist in separate but adjacent tumors [1][2][3][4]. While hyperprolactinemia due to pituitary stalk compression can occur, only rare cases have been described in the literature with the simultaneous presence of a prolactinoma and a sellar gangliocytoma [5,6].…”