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Infrasellar pituitary gangliocytoma causing Cushing’s syndrome

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Abstract

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.

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Acknowledgments

Marie-Eve Domingue received fellowship grants from Laval University (Bourse McLaughlin du Doyen, Québec, Canada), from EMD Serono, Canada and from Novo Nordisk, Belgium.

Conflict of interest

The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the report.

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Authors and Affiliations

  1. Department of Endocrinology and Nutrition, Cliniques Universitaires St-Luc, Avenue Hippocrate 10, 54.74, 1200, Brussels, Belgium

    Marie-Eve Domingue & Dominique Maiter

  2. Department of Pathology, Cliniques Universitaires St-Luc, Avenue Hippocrate 10, 1200, Brussels, Belgium

    Etienne Marbaix

  3. Regional Platform for Cell Imaging (PRIMACEN), Institute for Research and Innovation in Biomedicine (IRIB), University of Rouen, Mont-Saint-Aignan, France

    Jean-Luc Do Rego & Hubert Vaudry

  4. Division of Endocrinology, Clinique St-Pierre, 1300, Ottignies, Belgium

    Vincent Col

  5. Department of Neurosurgery, Cliniques Universitaires St-Luc, Avenue Hippocrate 10, 1200, Brussels, Belgium

    Christian Raftopoulos

  6. Department of Neuroradiology, Cliniques Universitaires St-Luc, Avenue Hippocrate 10, 1200, Brussels, Belgium

    Thierry Duprez

Authors
  1. Marie-Eve Domingue
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  2. Etienne Marbaix
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  3. Jean-Luc Do Rego
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  4. Vincent Col
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  5. Christian Raftopoulos
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  6. Thierry Duprez
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  7. Hubert Vaudry
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  8. Dominique Maiter
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Corresponding author

Correspondence to Dominique Maiter.

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Domingue, ME., Marbaix, E., Do Rego, JL. et al. Infrasellar pituitary gangliocytoma causing Cushing’s syndrome. Pituitary 18, 738–744 (2015). https://doi.org/10.1007/s11102-014-0595-4

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  • DOI: https://doi.org/10.1007/s11102-014-0595-4

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