title: Ectopic growth hormone-releasing hormone secretion by a metastatic bronchial carcinoid tumor: a case with a non hypophysial intracranial tumor that shrank during long acting octreotide treatment.
abstract: Ectopic acromegaly represents less than 1% of the reported cases of acromegaly. Although clinical improvement is common after treatment with somatostatin (SMS) analogs, the biochemical response and tumor size of the growth hormone-releasing hormone (GHRH)-producing tumor and its metastases are less predictable. Subject A 36-year-old male was referred because of a 3-year history of acromegaly related symptoms. He had undergone lung surgery in 1987 for a "benign" carcinoid tumor. Endocrine evaluation confirmed acromegaly Plasma IGF-1: 984 ng/ml (63-380), GH: 49.8 ng/ml (<5). MRI showed a large mass in the left cerebellopontine angle and diffuse pituitary hyperplasia. Pulmonary, liver and bone metastases were shown by chest and abdominal CT scans. Ectopic GHRH secretion was suspected. Methods Measurement of circulating GHRH levels by fluorescence immunoassay levels and immunohistochemical study of the primary lung tumor and metastatic tissue with anti-GHRH and anti-somatostatin receptor type 2 (sst2A) antibodies. Results Basal plasma GHRH: 4654 pg/ml (<100). Pathological study of liver and bone biopsy material and lung tissue removed 19 years earlier was consistent with an atypical carcinoid producing GHRH and exhibiting sst2A receptor expression. Treatment with octreotide LAR 20-40 mg q. month resulted in normalization of plasma IGF-1 levels. Circulating GHRH levels decreased dramatically. The size of the left prepontine cistern mass, with SMS receptors shown by a radiolabeled pentetreotide scan, decreased by 80% after 18 months of therapy. Total regression of pituitary enlargement was also observed. No changes were observed in lung and liver metastases. After 24 months of therapy the patient is asymptomatic and living a full and active life.
authors: Department of Endocrinology and Nuclear Medicine, Hospital Italiano, Gascón 450 (1187), Buenos Aires, Argentina. patricia.fainstein@hospitalitaliano.org.ar
authors: Patricia
authors: P
authors: Fainstein Day
authors: Lawrence
authors: L
authors: Frohman
authors: Hernan
authors: H
authors: Garcia Rivello
authors: Jean Claude
authors: JC
authors: Reubi
authors: Gustavo
authors: G
authors: Sevlever
authors: Mariela
authors: M
authors: Glerean
authors: Tomas
authors: T
authors: Fernandez Gianotti
authors: Marcelo
authors: M
authors: Pietrani
authors: Alejandra
authors: A
authors: Rabadan
authors: Silvina
authors: S
authors: Racioppi
authors: Martin
authors: M
authors: Bidlingmaier
mesh_terms: D000172:Acromegaly / Q000209:etiology; D000328:Adult; D018931:Antineoplastic Agents, Hormonal / Q000627:therapeutic use*; D001786:Blood Glucose / Q000378:metabolism; D001984:Bronchial Neoplasms / Q000188:drug therapy / Q000378:metabolism* / Q000556:secondary*; D002276:Carcinoid Tumor / Q000188:drug therapy / Q000378:metabolism* / Q000556:secondary*; D002530:Cerebellopontine Angle / Q000473:pathology; D013007:Growth Hormone-Releasing Hormone / Q000378:metabolism*; D006729:Hormones, Ectopic / Q000378:metabolism*; D019382:Human Growth Hormone / Q000378:metabolism*; D006801:Humans; D008099:Liver / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D015282:Octreotide / Q000627:therapeutic use*; D049268:Positron-Emission Tomography; D019275:Radiopharmaceuticals; D017481:Receptors, Somatostatin / Q000378:metabolism; D051598:Whole Body Imaging
chemical_list: D018931:Antineoplastic Agents, Hormonal; D001786:Blood Glucose; D006729:Hormones, Ectopic; D019275:Radiopharmaceuticals; D017481:Receptors, Somatostatin; D019382:Human Growth Hormone; D013007:Growth Hormone-Releasing Hormone; D015282:Octreotide