title: Ectopic acromegaly due to growth hormone releasing hormone.
abstract: Acromegaly secondary to extra-pituitary tumors secreting growth hormone releasing hormone (GHRH) is rarely encountered. We review the literature on ectopic acromegaly and present the index report of ectopic acromegaly secondary to GHRH secretion from a mediastinal paraganglioma. Clinical and pathological manifestations and therapeutic management of 99 patients with ectopic acromegaly are reviewed. Acromegaly secondary to ectopic GHRH secretion is usually caused by a neuroendocrine tumor in the lung and pancreas. We report an additional cause of ectopic acromegaly from a mediastinal paraganglioma. Diagnostic criteria of ectopic GHRH syndrome include biochemical and pathologic tumoral confirmation of GHRH secretion and expression. Management of ectopic acromegaly consists of surgical resection of the primary tumor and biochemical normalization, with possible adjuvant use of somatostatin analogs. The review demonstrates that there are several tumor types, including paragangliomas which may secrete GHRH, leading to acromegaly. Clinical and laboratory manifestations of the syndrome and challenges in diagnosis and management of these rarely encountered patients require early diagnosis and appropriate treatment to prevent long-term morbidity and mortality with ectopic acromegaly.
authors.affiliation: Endocrine Research Center, Research Institute for Endocrine Sciences, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
authors.forename: Ali A
authors.initials: AA
authors.lastname: Ghazi
authors.forename: Alireza
authors.initials: A
authors.lastname: Amirbaigloo
authors.forename: Azizollah Abbasi
authors.initials: AA
authors.lastname: Dezfooli
authors.forename: Navid
authors.initials: N
authors.lastname: Saadat
authors.forename: Siavash
authors.initials: S
authors.lastname: Ghazi
authors.forename: Marina
authors.initials: M
authors.lastname: Pourafkari
authors.forename: Farrokh
authors.initials: F
authors.lastname: Tirgari
authors.forename: Dheepti
authors.initials: D
authors.lastname: Dhall
authors.forename: Serguei
authors.initials: S
authors.lastname: Bannykh
authors.forename: Shlomo
authors.initials: S
authors.lastname: Melmed
authors.forename: Odelia
authors.initials: O
authors.lastname: Cooper
mesh_terms: D000172:Acromegaly / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology*; D000293:Adolescent; D000328:Adult; D000368:Aged; D003131:Combined Modality Therapy; D005260:Female; D013007:Growth Hormone-Releasing Hormone / Q000378:metabolism*; D006801:Humans; D008175:Lung Neoplasms / Q000378:metabolism / Q000601:surgery; D008297:Male; D008479:Mediastinal Neoplasms / Q000378:metabolism / Q000601:surgery; D008875:Middle Aged; D010190:Pancreatic Neoplasms / Q000378:metabolism / Q000601:surgery; D010236:Paraganglioma, Extra-Adrenal / Q000378:metabolism* / Q000601:surgery; D013004:Somatostatin / Q000031:analogs & derivatives / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D013004:Somatostatin; D013007:Growth Hormone-Releasing Hormone