title: Clinical characteristics and outcome of acromegaly induced by ectopic secretion of growth hormone-releasing hormone (GHRH): a French nationwide series of 21 cases.
abstract: BACKGROUND
  Ectopic GHRH secretion is a rare cause of acromegaly, and case reports are mainly isolated.
  
  
  METHODS
  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.
  
  
  METHODS
  Twenty-one patients aged 14-77 yr were identified from 12 French hospitals. Median GHRH was 548 (270-9779) ng/liter.
  
  
  METHODS
  Outcome measures included description of tumor features and outcome and the relation between plasma GHRH values and tumor site, size, and spread.
  
  
  RESULTS
  The primary neuroendocrine tumor was identified for 20 of 21 patients (12 pancreatic, seven bronchial, one appendicular). Tumors were large (10-80 mm), identified on computed tomography scan in 18 cases and by endoscopic ultrasound and somatostatin receptor scintigraphy in two. Somatostatin receptor scintigraphy had a similar sensitivity to computed tomography 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 yr, 85% of patients were alive. Ninety-one percent of patients whose tumor was completely removed were considered in remission, and most had normalized plasma GHRH. The remaining patients were treated with somatostatin analogs: IGF-I normalized except for one patient who required pegvisomant, but GHRH levels remained elevated. No correlations were found between GHRH levels and tumor site or size or the existence of metastases. Identification of increased plasma GHRH during follow-up was an accurate indicator of recurrence.
  
  
  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.
authors.affiliation: Université de Lyon, France.
authors.forename: Laetitia
authors.initials: L
authors.lastname: Garby
authors.forename: Philippe
authors.initials: P
authors.lastname: Caron
authors.forename: Francine
authors.initials: F
authors.lastname: Claustrat
authors.forename: Philippe
authors.initials: P
authors.lastname: Chanson
authors.forename: Antoine
authors.initials: A
authors.lastname: Tabarin
authors.forename: Vincent
authors.initials: V
authors.lastname: Rohmer
authors.forename: Gwenaëlle
authors.initials: G
authors.lastname: Arnault
authors.forename: Fabrice
authors.initials: F
authors.lastname: Bonnet
authors.forename: Olivier
authors.initials: O
authors.lastname: Chabre
authors.forename: Sophie
authors.initials: S
authors.lastname: Christin-Maitre
authors.forename: Hélène
authors.initials: H
authors.lastname: du-Boullay
authors.forename: Arnaud
authors.initials: A
authors.lastname: Murat
authors.forename: Ihab
authors.initials: I
authors.lastname: Nakib
authors.forename: Jean-Louis
authors.initials: JL
authors.lastname: Sadoul
authors.forename: Geneviève
authors.initials: G
authors.lastname: Sassolas
authors.forename: Bruno
authors.initials: B
authors.lastname: Claustrat
authors.forename: Gérald
authors.initials: G
authors.lastname: Raverot
authors.forename: Françoise
authors.initials: F
authors.lastname: Borson-Chazot
mesh_terms: D000172:Acromegaly / Q000209:etiology / Q000378:metabolism / Q000601:surgery; D000293:Adolescent; D000328:Adult; D000368:Aged; D001984:Bronchial Neoplasms / Q000150:complications / Q000378:metabolism / Q000601:surgery; D002276:Carcinoid Tumor / Q000150:complications / Q000378:metabolism / Q000601:surgery; D005260:Female; D005500:Follow-Up Studies; D005602:France; D013007:Growth Hormone-Releasing Hormone / Q000097:blood / Q000378:metabolism*; D049912:Growth Hormone-Secreting Pituitary Adenoma / Q000150:complications / Q000378:metabolism / Q000601:surgery; D006801:Humans; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000150:complications / Q000378:metabolism / Q000601:surgery; D018358:Neuroendocrine Tumors / Q000150:complications / Q000378:metabolism / Q000601:surgery; D010190:Pancreatic Neoplasms / Q000150:complications / Q000378:metabolism / Q000601:surgery; D010911:Pituitary Neoplasms / Q000150:complications / Q000378:metabolism / Q000601:surgery; D011379:Prognosis; D012042:Registries; D016896:Treatment Outcome
chemical_list: D013007:Growth Hormone-Releasing Hormone