title: Acromegaly caused by growth hormone-releasing hormone-producing tumors: long-term observational studies in three patients.
abstract: We report on three newly diagnosed patients with extracranial ectopic GHRH-associated acromegaly with long-term follow-up after surgery of the primary tumor. One patient with a pancreatic tumor and two parathyroid adenomas was the index case of a large kindred of MEN-I syndrome. The other two patients had a large bronchial carcinoid. The first patient is still in remission now almost 22 years after surgery. In the two other patients GHRH did not normalize completely after surgery and they are now treated with slow-release octreotide. IGF-I normalized in all patients. During medical treatment basal GH secretion remained (slightly) elevated and secretory regularity was decreased in 24 h blood sampling studies. We did not observe development of tachyphylaxis towards the drug or radiological evidence of (growing) metastases. We propose life-long suppressive therapy with somatostatin analogs in cases with persisting elevated serum GHRH concentrations after removal of the primary tumor. Independent parameters of residual disease are elevated basal (nonpulsatile) GH secretion and decreased GH secretory regularity.
authors.affiliation: Department of Endocrinology and Metabolic Diseases, Leiden University Medical Center, Albinusdreef 2, Leiden 2333 ZA, The Netherlands.
authors.forename: Nienke R
authors.initials: NR
authors.lastname: Biermasz
authors.forename: Jan W A
authors.initials: JW
authors.lastname: Smit
authors.forename: Alberto M
authors.initials: AM
authors.lastname: Pereira
authors.forename: Marijke
authors.initials: M
authors.lastname: Frölich
authors.forename: Johannes A
authors.initials: JA
authors.lastname: Romijn
authors.forename: Ferdinand
authors.initials: F
authors.lastname: Roelfsema
mesh_terms: D000172:Acromegaly / Q000209:etiology* / Q000473:pathology; D000236:Adenoma / Q000378:metabolism* / Q000473:pathology / Q000601:surgery; D000328:Adult; D002276:Carcinoid Tumor / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000601:surgery; D019277:Entropy; D005260:Female; D006728:Hormones / Q000097:blood; D019382:Human Growth Hormone / Q000378:metabolism*; D006801:Humans; D008137:Longitudinal Studies; D008175:Lung Neoplasms / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000601:surgery; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D015282:Octreotide; D010190:Pancreatic Neoplasms / Q000378:metabolism* / Q000473:pathology / Q000601:surgery; D009384:Paraneoplastic Endocrine Syndromes / Q000378:metabolism* / Q000473:pathology / Q000601:surgery; D010282:Parathyroid Neoplasms / Q000378:metabolism* / Q000473:pathology / Q000601:surgery; D010902:Pituitary Gland / Q000473:pathology; D049268:Positron-Emission Tomography; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D006728:Hormones; D019382:Human Growth Hormone; D015282:Octreotide