Horm Metab Res 2011; 43(4): 287-291
DOI: 10.1055/s-0030-1270523
Humans, Clinical

© Georg Thieme Verlag KG Stuttgart · New York

Transient Efficacy of Octreotide and Pasireotide (SOM230) Treatment in GIP-dependent Cushing's Syndrome

V. Preumont1 , L. M. Mermejo2 , P. Damoiseaux3 , A. Lacroix2 , D. Maiter1
  • 1Division of Endocrinology, Cliniques Universitaires Saint-Luc UCL, Brussels, Belgium
  • 2Division of Endocrinology, Department of Medicine, Centre Hospitalier de l’Université de Montréal, Montréal, Canada
  • 3Division of Endocrinology, Centre Hospitalier de Dinant, Dinant, Belgium
Further Information

Publication History

Publication Date:
24 January 2011 (online)

Abstract

We studied a 55-year old woman presenting with features of Cushing's syndrome associated with metabolic abnormalities including severe hypertension and type 2 diabetes. Urinary free cortisol excretion was within normal limits, but an unusual diurnal cortisol rhythm was observed with low morning and high postprandial levels, associated with the absence of cortisol suppression after dexamethasone, suggesting the possibility of GIP-dependent Cushing's syndrome. The diagnosis was confirmed by further investigations, showing significant plasma cortisol responses after a mixed meal test and after oral, but not intravenous glucose administration, as well as ACTH-independent bilateral macronodular adrenal hyperplasia (AIMAH). An aberrant increase in cortisol was also observed after glucagon and terlipressin injections. The patient was first treated with octreotide 100–250 μg thrice daily for 6 months, then with the new multi-ligand somatostatin analogue (SOM 230) 450–900 μg twice daily for 3 months. Although inducing a significant acute suppression of post-prandial cortisol response, both drugs had no effects on the clinical and metabolic abnormalities associated with Cushing's syndrome and new tests performed at the end of each treatment period confirmed escape of post-meal cortisol suppression to therapy. The patient finally underwent a bilateral adrenalectomy, which markedly improved her medical condition and allowed in vitro confirmation by real time RT-PCR quantification of a high aberrant expression of GIP receptor mRNA in adrenal tissue. This case report illustrates the lack of sustained efficacy of somatostatin analogues on GIP-dependent Cushing's syndrome, independent of their affinity for the different somatostatin receptor subtypes.

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Correspondence

Dr. V.Preumont 

Division of Endocrinology

Cliniques Universitaires

UCL Saint-Luc

Avenue Hippocrate, 10.

UCL 5474

1200 Brussels

Belgium

Phone: +32/2/764 54 75

Fax: +32/2/764 54 18

Email: vanessa.preumont@uclouvain.be

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