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Infrasellar pituitary gangliocytoma causing Cushing's syndrome

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Domingue, Marie-Eve [UCL] Marbaix, Etienne [UCL] Do Rego, Jean-Luc Col, Vincent Raftopoulos, Christian [UCL] Duprez, Thierry [UCL] Maiter, Dominique [UCL]

INTRODUCTION: Pituitary gangliocytomas are uncommon neuronal tumours that may present with endocrine disorders, the most frequent being acromegaly caused by growth hormone hypersecretion. Cushing's syndrome is very rarely seen with gangliocytomas. MATERIAL AND METHODS: We report the unique case of a 62 year-old woman whose clinical picture and endocrine testing clearly demonstrated adrenocorticotropin (ACTH)-dependent Cushing's syndrome. Pituitary magnetic resonance imaging showed a 12-mm homogeneous, infra- and retrosellar mass first diagnosed as pituitary macroadenoma. Transsphenoidal surgery was performed and allowed complete resection of the tumour with sparing of normal anterior pituitary. Very low postoperative serum cortisol and ACTH levels were observed in the early postoperative period and the patient is still in remission 18 months after surgery, thus demonstrating that the resected lesion was entirely responsible for the clinical picture. RESULTS: Histological and immunocytochemical analyses demonstrated a benign tumour composed of mature neuronal cells suggestive of a gangliocytoma, expressing both ACTH and corticotropin-releasing hormone (CRH). The tumour was surrounded by a rim of pituitary tissue containing ACTH-producing endocrine cells. Careful analysis of the resected lesion did not reveal any pituitary microadenoma. We search literature for similar cases and retraced only nine cases of gangliocytomas associated with Cushing's syndrome. In most of them, the tumour was combined with either pituitary corticotroph adenoma or hyperplasia. CONCLUSIONS: Our case represents a unique case of an infrasellar pituitary gangliocytoma which was able to cause Cushing's syndrome by both direct ACTH production and CRH-induced stimulation of neighbour normal corticotroph cells.

Document type Article de périodique (Journal article) – Synthèse de littérature
Access type Accès restreint
Publication date 2015
Language Anglais
Journal information "Pituitary" - Vol. 18, no. 5, p. 738-744 (2015)
Peer reviewed yes
Publisher Springer New York LLC ((United States) New York, NY)
issn 1386-341X
e-issn 1573-7403
Publication status Publié
Affiliations UCL - SSS/IONS/NEUR - Clinical Neuroscience
UCL - SSS/IREC/EDIN - Pôle d'endocrinologie, diabète et nutrition
UCL - (SLuc) Service d'endocrinologie et de nutrition
UCL - (SLuc) Service de radiologie
UCL - (SLuc) Service d'anatomie pathologique
UCL - SSS/DDUV/CELL - Biologie cellulaire
UCL - (SLuc) Service de neurochirurgie
Links
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Bibliographic reference Domingue, Marie-Eve ; Marbaix, Etienne ; Do Rego, Jean-Luc ; Col, Vincent ; Raftopoulos, Christian ; et. al. Infrasellar pituitary gangliocytoma causing Cushing's syndrome. In: Pituitary, Vol. 18, no. 5, p. 738-744 (2015)
Permanent URL http://hdl.handle.net/2078.1/150567