Intrasellar gangliocytoma and syndromes of pituitary hypersecretion

Abstract

Syndromes of hypersecretion of pituitary hormone and sellar enlargement may on occasion be caused by a gangliocytoma instead of a pituitary adenoma. At least some of these rare tumors are apparently independent of and separable from the pituitary gland, its stalk, and the hypothalamus, and are therefore surgically removable without incurring further endocrine deficit. The authors report such a case, with successful removal of the tumor via a frontal craniotomy. The associated hypersecretion of pituitary hormone was corrected without disturbing normal pituitary function.

Sellar Neuroblastoma Mimicking Pituitary Adenoma

“…The earlier pathological diagnosis should have been confirmed by immunohistochemicaland ultrastructural studies when the tumor recurred within only 16 months, which reflected its malignant potential. Intrasellar neuronal neoplasms are very uncommon, they usually are well-differentiated gangliocytomas or ganglioneuromas [10][11][12][13][14][15][16], in the middle of a spectrum of tumors between neuronal hamartomas and rare malignant gangliogliomas [17]. In our patients, histological study returned a diagnosis of neuroblastoma; the tumor exhibited no features of gangliocytoma such as binucleated ganglial cells, glial stroma, or calcospherites.…”

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

“…Sellar gangliocytomas may cause hypersecretion of pituitary hormone and enlargement of the sella turcica. Sometimes, they are separable from the pituitary gland, stalk, and hypothalamus, and may be successfully removed without further endocrine deficit [16]. Most sellar ganglion cell lesions are related to functioning or nonfunctioning pituitary adenomas or pituitary cell hyperplasia.…”

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

“…Secretion of hormones by the ganglionic cells of hypothalamic origin could lead to the development of adenomas. However, this hypothesis was contradicted because of a lack of correlation between the released hypothalamic hormones and the adenoma cell secretions in some cases ( 12 , 27 ). Moreover, the hypothalamic-releasing hormones secreted by the ganglionic cells seem to promote proliferation and not initiate tumorigenesis of pituitary adenomas.…”

“…Ganglion cell-containing tumors of the pituitary gland have been reported under various names ( 4 , 6 , 11 ). Several authors have reviewed pituitary GCs, including MGAs, under the former diagnoses of “gangliocytoma,” “ganglioneuroma,” “pituitary adenoma with neuronal choristoma,” “ganglioneuroma and adenoma,” “gangliocytoma with adenoma,” “pituitary adenoma with gangliocytic differentiation,” “adenohypophyseal neuronal choristoma,” and others ( 2–5 , 7 , 12 ).…”