Food-dependent Cushing’s syndrome due to unilateral adrenocortical adenoma with cortisol secretion without ACTH elevation detected in peripheral blood by the CRH test: a case report

We report an extremely rare case of a 61-year old woman with food-dependent Cushing’s syndrome (FDC) due to unilateral adrenocortical adenoma (UAA) with cortisol (CORT) secretion without ACTH elevation detected in peripheral blood by the CRH test. She was on oral medications for hypertension and dep...

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Bibliographic Details
Published in:Endocrine Journal 2023, Vol.70(7), pp.745-753
Main Authors: Makino, Miwa, Koriyama, Nobuyuki, Kojima, Nami, Tobo, Takuya, Nishio, Yoshihiko
Format: Article
Language:English
Subjects:
Adenoma
Adrenalectomy
Adrenocorticotropic hormone
Case reports
Cortisol
CRH
CRH receptor 2
Diurnal variations
Food-dependent Cushing’s syndrome
GIP protein
Glucose
Glucose tolerance
Glucose-dependent insulinotropic polypeptide
Hormones
Hydrocortisone
Hypokalemia
Intravenous administration
Laparoscopy
Nervous system diseases
Peripheral blood
Pituitary
Tumors
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Summary:We report an extremely rare case of a 61-year old woman with food-dependent Cushing’s syndrome (FDC) due to unilateral adrenocortical adenoma (UAA) with cortisol (CORT) secretion without ACTH elevation detected in peripheral blood by the CRH test. She was on oral medications for hypertension and depression, and presented weight gain, general fatigue, muscle weakness, and hypokalemia. Despite the fact that the diurnal variation of ACTH was always suppressed, a diurnal variation in CORT was observed, in the form of low levels in the early morning and high levels in the afternoon. An increase in CORT was shown in a 75 g-oral glucose tolerance test (OGTT) and in a mixed meal tolerance test, but no change in CORT levels was seen in intravenous glucose tolerance tests. Elevated CORT levels were observed in response to intravenous injection of CRH, although ACTH levels were always below the measured sensitivity. Laparoscopic left adrenalectomy was performed, which resulted in postoperative improvement in potassium and ACTH levels and disappearance of the CORT secretory response in the OGTT. Clear expression of glucose-dependent insulinotropic polypeptide receptor (GIPR), CRH and CRH receptor 2 (CRHR2) were confirmed in the surgically-resected UAA specimen by molecular and immunohistochemical analyses, suggesting the involvement of not only GIPR, but also CRH and CRHR2 in FDC.
ISSN:0918-8959
1348-4540
DOI:10.1507/endocrj.EJ22-0622