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Table of Contents    
Year : 2012  |  Volume : 60  |  Issue : 3  |  Page : 311-313

Combined gangliocytoma and non-functioning pituitary adenoma of the pituitary gland

Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu, Sichuan Province, 610041, China

Date of Submission27-Feb-2012
Date of Decision15-Mar-2012
Date of Acceptance28-Apr-2012
Date of Web Publication14-Jul-2012

Correspondence Address:
Shu Jiang
Department of Neurosurgery, West China Hospital, Sichuan University, Chengdu, Sichuan Province, 610041
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.98522

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How to cite this article:
Zhou P, Ma L, Cheng S, Yin S, Jiang S. Combined gangliocytoma and non-functioning pituitary adenoma of the pituitary gland. Neurol India 2012;60:311-3

How to cite this URL:
Zhou P, Ma L, Cheng S, Yin S, Jiang S. Combined gangliocytoma and non-functioning pituitary adenoma of the pituitary gland. Neurol India [serial online] 2012 [cited 2023 Dec 7];60:311-3. Available from:


The co-existence of neural tissue and pituitary adenoma in the sella turcica is very rare. To date, no more than 75 cases have been reported. [1],[2],[3],[4] They often present with hypersecretion of growth hormone (GH), prolactin and adrenocorticotrophic hormone. Few sporadic cases have been described in the literature with the simultaneous presence of non-functioning adenomatous cells and ganglion cells. [3],[4],[5],[6],[7],[8],[9],[10] We report a case of this tumor type and propose our hypothesis about the origin of this combined tumor.

A 45-year-old woman was admitted with a 2-year history of menstrual disorder and a 5-month history of headache. Basal levels of pituitary hormones were all normal. Magnetic resonance imaging (MRI) revealed a slightly enhanced sellar lesion with 21 mm at the largest diameter [Figure 1]a-c. The grey and leathery mass [Figure 1]d was subtotally removed via the transsphenoidal approach. Post-operatively, there were no complications and the headache subsided. Histopathology identified the co-existence of non-functioning adenoma and gangliocytoma. Histology revealed two kinds of co-existing tumor cell areas [Figure 2]a and b. The typical and diffused adenoma cells were spherical to ovoid, uniformly large and polyhedral. The large scattered immature multipolar gangliocytoma cells are haphazardly arranged, with basophilic cytoplasm and prominent nucleoli, with Nissl substance in the cytoplasm processes. Immunohistochemical staining revealed that the adenomatous tissue was negative for all pituitary hormones and positive for CgA. Some gangliocytoma cells were identified by positive neurofilament protein, neuron-specific enolase and CD133 staining [Figure 2]c and d and negative glial fibrillary acidic protein. The 3-month post-operative contrast MRI scan revealed a small residual lesion and a gamma knife radiosurgery was performed. No enlargement of the residual tumor was detected during the following 14-month follow-up.
Figure 1: Magnetic resonance imaging (MRI) shown a sella lesion. Tumor on MRI (a– c, white arrows) and surgical finding (d)

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Figure 2: Hematoxylin and eosin staining of the tumor shows that the adenomatous cells (white arrows) and the gangliocytic cells (black arrows) formed separate and intermingled areas (a and b) Only the bigger gangliocytic cells were weakly positive for CD133 by immunohistochemical staining (c and d, black arrows)

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Gangliocytoma associated with pituitary adenoma is a rare tumor composed of both adenomatous and gangliocytic elements. This combined tumor accounts for 0.25-1.26% of sellar tumors. [3],[4] Most of them were women and diagnosed as GH-secreting pituitary adenoma pre-operatively, while, in a few cases, the tumor presented with symptoms of Cushing's disease and hyperprolactinemia. [4],[5],[8],[9],[10] Reports of gangliocytoma in combination with non-functioning adenoma are scarce. [1] The definitive diagnosis is by histological studies.

The origin of these tumors is controversial, and three main hypotheses have been proposed. The first hypothesis states that such tumor results from the co-incidence of pituitary adenoma and abnormal migration of hypothalamic neurons within the adenohypophysial parenchyma during the early phase of embryogenesis. Another hypothesis is that the pituitary adenoma is caused by excessive stimulation of the hypothalamic hormones from the heterotopic intrasellar ganglion cells. [2],[7] However, the lack of correlation between the adenoma type and the hypothalamic hormone, as in our patient, is not in favor of this hypothesis. The third hypothesis suggests that the neuronal and adenomatous component originated from the transitional cell between the neurons and adenohypophysial cells of the embryonic rests; [11] the presence of intermediate cells, [12] ependymal cells and nerve bundle structure support this hypothesis. [6],[13] It is known that the anterior pituitary develops from Rathke's pouch, which is an outgrowth of the embryonic mouth, and the posterior lobe develops from the base of the embryonic brain as it grows down to meet the developing Rathke's pouch. As the glandular elements and the gangliocytic elements are allelotaxis, the gangliocytic cells seldom migrate to the anterior lobe. In our case, the neuronal compartment of this tumor was observed to be admixed or separate from the adenoma elements. Based on the character of our case and the literature review, we partly agree with the first and the third hypotheses that this type of tumor may relate to the embryonic cells. But, the details of this tumorigenesis are arguable. The weakly positive immunohistochemical staining for CD133 and the morphological characters of the gangliocytoma cells prompts that it may be immature; those two elements may be of different differentiation stage. To begin with, this collision lesion may develop from the abnormal migration of gangliocytic cells in the embryonic brain due to internal or external disturbance. Then, the ectopic ganglion cells keep the immature features in abnormal location and lead to the tumorigenesis of the pituitary adenoma. If cells can be isolated and cultured from the specimen of this type of tumor, it would endow us with the ability to understand this collision lesion and the pituitary tumor better.

 » Acknowledgment Top

The additional immunohistochemical staining was supported by the National Natural Science Foundation of China (81072077) and Foundation of Science and Technology Department of Sichuan Provience (2010SZ0160)

 » References Top

1.Andújar-Plata P, Cabezas-Agrícola JM, Rivero-Luis MT, Pérez-Becerra E, García-Allut A, Casanueva FF. Non-functioning gangliocytoma of the pituitary gland. Endocrinol Nutr 2011;58:52-3.  Back to cited text no. 1
2.Kontogeorgos G, Mourouti G, Kyrodimou E, Liapi-Avgeri G, Parasi E. Ganglion cell containing pituitary adenomas: Signs of neuronal differentiation in adenoma cells. Acta Neuropathol 2006;112:21-8.  Back to cited text no. 2
3.Koutourousiou M, Kontogeorgos G, Wesseling P, Grotenhuis AJ, Seretis A. A collision sellar lesions: Experience with eight cases and review of the literature. Pituitary 2010;13:8-17.  Back to cited text no. 3
4.Kurosaki M, Saeger W, Lüdecke DK. Intrasellar gangliocytomas associated with acromegaly. Brain Tumor Pathol 2002;19:63-7.  Back to cited text no. 4
5.Mikami S, Kameyama K, Takahashi S, Yoshida K, Kawase T, Sano T, et al. Combined gangliocytoma and prolactinoma of the pituitary gland. Endocr Pathol 2008;19:117-21.  Back to cited text no. 5
6.Xiao P, Xue L, Peng JJ, Feng ST, Liao B, Wen JM. An intrasellar mixed gangliocytoma-adenoma including ependymal component, and review of the literature. BMJ Case Rep 2009;2009. doi: 10.1136/bcr.11.2008.1200.  Back to cited text no. 6
7.Puchner MJ, Lüdecke DK, Valdueza JM, Saeger W, Willig RP, Stalla GK, et al. Cushing's disease in a child caused by a corticotropin-releasing hormone-secreting intrasellar gangliocytoma associated with an adrenocorticotropic hormone-secreting pituitary adenoma. Neurosurgery 1993;33:920-5.  Back to cited text no. 7
8.Saeger W, Puchner MJ, Lüdecke DK. Combined sellar gangliocytoma and pituitary adenoma in acromegaly or Cushing's disease: A report of three cases. Virchows Arch 1994;425:93-9.  Back to cited text no. 8
9.Serri O, Berthelet F, Bélair M, Vallette S, Asa SL. An unusual association of a sellar gangliocytoma with a prolactinoma. Pituitary 2008;11:85-7.  Back to cited text no. 9
10.Thodou E, Kontogeorgos G, Horvath E, Kovacs K. Prolactin-producing pituitary adenoma with incomplete neuronal transformation: An intermediate adenoma-neuronal tumor. Acta Neuropathol 2004;108:115-20.  Back to cited text no. 10
11.Horvath E, Kovacs K, Scheithauer BW, Lloyd RV, Smyth HS. Pituitary adenoma with neuronal choristoma (PANCH): Composite lesion or lineage infidelity? Ultrastruct Pathol 1994;18:565-74.  Back to cited text no. 11
12.Vidal S, Horvath E, Bonert V, Shahinian K, Kovacs K. Neural transformation in pituitary corticotroph adenoma. Acta Neuropathol 2002;104:435-40.  Back to cited text no. 12
13.Ikeda H, Takayasu S. A growth hormone-secreting adenoma with incomplete nerve bundle formation. Neuropathology 2008;28:317-21.  Back to cited text no. 13


  [Figure 1], [Figure 2]

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