학술논문
Pituitary MRI Features in Acromegaly Resulting From Ectopic GHRH Secretion From a Neuroendocrine Tumor: Analysis of 30 Cases
Clinical Research Article
Clinical Research Article
Document Type
Academic Journal
Author
Potorac, Iulia; Bonneville, Jean-Francois; Daly, Adrian F.; Herder, Wouter de; Fainstein-Day, Patricia; Chanson, Philippe; Korbonits, Marta; Cordido, Fernando; Lamback, Elisa Baranski; Abid, Mohamed; Raverot, Veronique; Raverot, Gerald; Caron, Philippe; Boullay, Helene Du; Bidlingmaier, Martin; Bolanowski, Marek; Laloi-Michelin, Marie; Borson-Chazot, Francoise; Chabre, Olivier; Briet, Claire; Diaz-Soto, Gonzalo; Bonneville, Fabrice; Castinetti, Frederic; Gadelha, Monica R.; Santana, Nathalie Oliveira; Stelmachowska-Banas, Maria; Gudbjartsson, Tomas; Villar-Taibo, Rocio; Zornitzki, Taiba; Tshibanda, Luaba; Petrossians, Patrick; Beckers, Albert
Source
Journal of Clinical Endocrinology & Metabolism. August 2022, Vol. 107 Issue 8, pe3313, 8 p.
Subject
Language
English
ISSN
0021-972X
Abstract
Acromegaly is a rare endocrine disorder with an estimated prevalence of 10.5 cases per 100 000 individuals (1). It is usually the result of GH hypersecretion from a pituitary adenoma. [...]
Context: Ectopic acromegaly is a consequence of rare neuroendocrine tumors (NETs) that secrete GHRH. This abnormal GHRH secretion drives GH and IGF-1 excess, with a clinical presentation similar to classical pituitary acromegaly. Identifying the underlying cause for the GH hypersecretion in the setting of ectopic GHRH excess is, however, essential for proper management both of acromegaly and the NET. Owing to the rarity of NETs, the imaging characteristics of the pituitary in ectopic acromegaly have not been analyzed in depth in a large series. Objective: Characterize pituitary magnetic resonance imaging (MRI) features at baseline and after NET treatment in patients with ectopic acromegaly. Design: Multicenter, international, retrospective. Setting: Tertiary referral pituitary centers. Patients: Thirty ectopic acromegaly patients having GHRH hypersecretion. Intervention: None. Main outcome measure: MRI characteristics of pituitary gland, particularly T2-weighted signal. Results: In 30 patients with ectopic GHRH-induced acromegaly, we found that most patients had hyperplastic pituitaries. Hyperplasia was usually moderate but was occasionally subtle, with only small volume increases compared with normal ranges for age and sex. T2-weighted signal was hypointense in most patients, especially in those with hyperplastic pituitaries. After treatment of the NET, pituitary size diminished and T2-weighted signal tended to normalize. Conclusions: This comprehensive study of pituitary MRI characteristics in ectopic acromegaly underlines the utility of performing T2-weighted sequences in the MRI evaluation of patients with acromegaly as an additional tool that can help to establish the correct diagnosis. Key Words: acromegaly, ectopic, MRI, GHRH, T2-hypointense, pituitary, neuroendocrine tumor Abbreviations: CV, coefficient of variation; MEN1, multiple endocrine neoplasia type 1; MRI, magnetic resonance imaging; NET, neuroendocrine tumor; SRL, somatostatin receptor ligand; T2W, T2-weighted
Context: Ectopic acromegaly is a consequence of rare neuroendocrine tumors (NETs) that secrete GHRH. This abnormal GHRH secretion drives GH and IGF-1 excess, with a clinical presentation similar to classical pituitary acromegaly. Identifying the underlying cause for the GH hypersecretion in the setting of ectopic GHRH excess is, however, essential for proper management both of acromegaly and the NET. Owing to the rarity of NETs, the imaging characteristics of the pituitary in ectopic acromegaly have not been analyzed in depth in a large series. Objective: Characterize pituitary magnetic resonance imaging (MRI) features at baseline and after NET treatment in patients with ectopic acromegaly. Design: Multicenter, international, retrospective. Setting: Tertiary referral pituitary centers. Patients: Thirty ectopic acromegaly patients having GHRH hypersecretion. Intervention: None. Main outcome measure: MRI characteristics of pituitary gland, particularly T2-weighted signal. Results: In 30 patients with ectopic GHRH-induced acromegaly, we found that most patients had hyperplastic pituitaries. Hyperplasia was usually moderate but was occasionally subtle, with only small volume increases compared with normal ranges for age and sex. T2-weighted signal was hypointense in most patients, especially in those with hyperplastic pituitaries. After treatment of the NET, pituitary size diminished and T2-weighted signal tended to normalize. Conclusions: This comprehensive study of pituitary MRI characteristics in ectopic acromegaly underlines the utility of performing T2-weighted sequences in the MRI evaluation of patients with acromegaly as an additional tool that can help to establish the correct diagnosis. Key Words: acromegaly, ectopic, MRI, GHRH, T2-hypointense, pituitary, neuroendocrine tumor Abbreviations: CV, coefficient of variation; MEN1, multiple endocrine neoplasia type 1; MRI, magnetic resonance imaging; NET, neuroendocrine tumor; SRL, somatostatin receptor ligand; T2W, T2-weighted