The Ectopic Adrenocorticotropic Hormone Syndrome: Rarely Easy, Always Challenging

Endocrinology and Metabolism Clinics of North America
Aimee R Hayes, Ashley B Grossman

Abstract

Despite modern imaging techniques, differentiating ectopic adrenocorticotropic hormone (ACTH) syndrome from pituitary-dependent Cushing's syndrome, Cushing's disease, is especially difficult when well-differentiated carcinoids are the source of ACTH secretion, particularly pulmonary carcinoid tumors. ACTH-secreting pulmonary carcinoids, like the corticotroph adenomas causing Cushing's disease, are often small and difficult to detect, and patients present with a gradual onset of the classical signs and symptoms of Cushing's syndrome, indistinguishable from the presentation of Cushing's disease. Hence, the differential diagnosis relies on a combination of clinical assessment, dynamic biochemical tests, inferior petrosal sinus sampling, and multimodal imaging, each with its own caveats and pitfalls.

Citations

Jul 30, 2019·American Journal of Hypertension·Nattawat KlomjitSandra J Taler
Oct 31, 2019·The Journal of Clinical Endocrinology and Metabolism·German RubinsteinMartin Reincke
Apr 7, 2020·Current Opinion in Endocrinology, Diabetes, and Obesity·Seda GrigoryanAdina F Turcu
Jun 12, 2019·World Journal of Clinical Cases·Tong ZhouGui-Xia Wang
Jul 1, 2020·The Journal of Clinical Endocrinology and Metabolism·Caroline FreteAntoine Tabarin
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May 8, 2021·Reviews in Endocrine & Metabolic Disorders·Fernando Guerrero-PérezCarles Villabona
May 27, 2021·Endocrine·Sanna ToivanenCamilla Schalin-Jäntti
Sep 30, 2021·Experimental and Therapeutic Medicine·Adina GhemigianAna Valea
Jan 15, 2022·Archives of Endocrinology and Metabolism·Fernanda F AntonacioGilberto de Castro

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