The clinical and biochemical spectrum of ectopic acromegaly.

Autor: Fainstein-Day P; Endocrinology, Metabolism and Nuclear Medicine Department, Hospital Italiano de Buenos Aires (HIBA), Instituto de Medicina Traslacional e Ingeniería Biomédica (IMTIB), Consejo Nacional de Investigaciones Científicas y Técnicas (CONICET), Instituto Universitario del Hospital Italiano de Buenos Aires, Tte. Gral. Juan Domingo Perón 4190, Buenos Aires, CP C1199ABB, Argentina. Electronic address: patricia.fainstein@hospitalitaliano.org.ar., Ullmann TE; Endocrinology, Metabolism and Nuclear Medicine Department, Hospital Italiano de Buenos Aires (HIBA), Tte. Gral. Juan Domingo Perón 4190, Buenos Aires, CP C1199ABB, Argentina. Electronic address: tamara.ullmann@hospitalitaliano.org.ar., Dalurzo MCL; Pathology Department, Hospital Italiano de Buenos Aires (HIBA), Tte. Gral. Juan Domingo Perón 4190, Buenos Aires, CP C1199ABB, Argentina. Electronic address: mercedes.dalurzo@hospitalitaliano.org.ar., Sevlever GE; Department of Neuropathology and Molecular Biology. Fundación para la Lucha contra las Enfermedades Neurológicas de la Infancia (FLENI), Montañeses 2325, C1428AQK Buenos Aires, Argentina. Electronic address: gsevlever@fleni.org.ar., Smith DE; General Surgery Department, Hospital Italiano de Buenos Aires (HIBA), Argentina. Electronic address: david.smith@hospitalitaliano.org.ar.
Jazyk: angličtina
Zdroj: Best practice & research. Clinical endocrinology & metabolism [Best Pract Res Clin Endocrinol Metab] 2024 May; Vol. 38 (3), pp. 101877. Date of Electronic Publication: 2024 Feb 14.
DOI: 10.1016/j.beem.2024.101877
Abstrakt: Ectopic acromegaly is a rare condition caused by extrapituitary central or peripheral neuroendocrine tumours (NET) that hypersecrete GH or, more commonly, GHRH. It affects less than 1% of acromegaly patients and a misdiagnosis of classic acromegaly can lead to an inappropriate pituitary surgery. Four types of ectopic acromegaly have been described: 1) Central ectopic GH-secretion: Careful cross-sectional imaging is required to exclude ectopic pituitary adenomas. 2) Peripheral GH secretion: Extremely rare. 3) Central ectopic GHRH secretion: Sellar gangliocytomas immunohistochemically positive for GHRH are found after pituitary surgery. 4) Peripheral GHRH secretion: The most common type of ectopic acromegaly is due to peripheral GHRH-secreting NETs. Tumours are large and usually located in the lungs or pancreas. Pituitary hyperplasia resulting from chronic GHRH stimulation is difficult to detect or can be misinterpreted as pituitary adenoma in the MRI. Measurement of serum GHRH levels is a specific and useful diagnostic tool. Surgery of GHRH-secreting NETs is often curative.
(Copyright © 2024 Elsevier Ltd. All rights reserved.)
Databáze: MEDLINE
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