ICEECE2012 Poster Presentations Growth hormone IGF axis - basic (23 abstracts)
Clinical characteristics and outcome of acromegaly induced by ectopic secretion of GHRH: a French nationwide series of 21 cases
L. Garby 1 , P. Caron 2 , F. Claustrat 1 , P. Chanson 3 , A. Tabarin 5 , V. Rohmer 6 , G. Arnault 7 , F. Bonnet 8 , O. Chabre 9 , S. Christin Maitre 4 , H. Du Boullay 10 , A. Murat 11 , I. Nakib 12 , J. Sadoul 13 , G. Sassolas 1 , B. Claustrat 1 , G. Raverot 1 & F. Borson Chazot 1
1Hopitaux de lyon, Groupement Hospitalier Est, Lyon, France; 2Hopital Larray, CHU Toulouse, Toulouse, France; 3Hopital du Kremlin Bicêtre, Assistance publique – Hôpitaux de Paris, Paris, France; 4Hopital Saint Antoine, Assistance publique – Hôpitaux de Paris, Paris, France; 5Hopital Haut Levêque, CHU Bordeaux, Bordeaux, France; 6Hopital Universitaire d’Angers, Angers, France; 7Centre hospitalier général de Vannes, Vannes, France; 8Hopital Anne de Bretagne, CHU Rennes, Rennes, France; 9Hopital de la Tronche, CHU Grenoble, Grenoble, France; 10Hopital de Chambéry, Chambéry, France; 11Hopital Nord Laennec, CHU Nantes, Nantes, France; 12Clinique Courlancy, Reims, France; 13Hopital L’Archet, CHU Nice, Nice, France.
Context: Ectopic GHRH secretion is a rare cause of acromegaly described mainly in isolated case reports.
Setting: From the registry of the sole laboratory performing plasma GHRH assays in France, we identified cases of ectopic GHRH secretion presenting with acromegaly between 1983 and 2008.
Patients: In total, 21 patients aged 14–77 years, from 12 French hospitals. Median GHRH was 548 ng/l (270–9779).
Main Outcome Measures: Description of tumor features and outcome, relation between plasma GHRH values and tumor site, size and spread.
Results: The primary neuroendocrine tumor was identified for 20/21 patients (12 pancreatic, seven bronchial, one appendicular). Tumors were large (10–80 mm), identified on CT scan in 18 cases and by endoscopic ultrasound and somatostatin receptor scintigraphy (SRS) in two cases. SRS had a similar sensitivity to CT scan (81 vs 86%). Tumors were all well-differentiated; 47.6% had metastasized at the time of diagnosis of acromegaly. After a median follow-up of 5 years, 85% of patients were alive. About 91 percent of patients whose tumor was completely removed were considered in remission and most had normalized plasma GHRH. Remaining patients were treated with somatostatin analogs: IGF1 normalized except for one patient who required pegvisomant, but GHRH levels remained elevated. No correlations were found between GHRH levels and tumor site, tumor size nor the existence of metastases. Determination of plasma GHRH during follow-up was an accurate indicator of recurrences.
Conclusions: The prognosis of endocrine tumors responsible for GHRH secretion appears relatively good. Plasma GHRH assay is an accurate tool for diagnosis and follow-up.
Declaration of interest: The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research project.
Funding: This work was supported, however funding details unavailable.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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