A rare cause of severe Cushing’s syndrome
Author(s)
Type
Journal Article
Abstract
Ectopic adrenocorticotropic hormone (ACTH) production is an uncommon cause of Cushing’s syndrome and rarely, the source can be a phaeochromocytoma. A 55-year-old man presented following an episode of presumed gastroenteritis with vomiting and general malaise. Further episodes of diarrhoea, joint pains and palpitations followed. On examination, he was hypertensive with no clinical features to suggest hypercortisolaemia. He was subsequently found to have raised plasma normetanephrines of 3.98 nmol/l (NR <0.71) and metanephrines of 0.69 nmol/l (NR <0.36). An adrenal CT showed a 3.8 cm right adrenal nodule, which was not MIBG-avid but was was clinically and biochemically consistent with a phaeochromocytoma. He was started on alpha blockade and referred for right adrenalectomy. Four weeks later, on the day of admission for adrenalectomy, profound hypokalaemia was noted (serum potassium 2.0 mmol/L) with non-specific ST-segment ECG changes. He was also diagnosed with new-onset diabetes mellitus (capillary blood glucose of 28 mmol/L). He reported to have gained weight and his skin had become darker over the course of the last four weeks. Given these findings, he underwent overnight dexamethasone suppression testing, which showed a non-suppressed serum cortisol of 1099 nmol/L. Baseline serum ACTH was 273 ng/L. A preliminary diagnosis of ectopic ACTH secretion from the known right-sided phaeochromocytoma was made and he was started on metyrapone and insulin. Surgery was postponed for 4 weeks. Following uncomplicated laparoscopic adrenalectomy, the patient recovered with full resolution of symptoms.
Date Issued
2020-03
Date Acceptance
2020-02-25
Citation
Endocrinology, Diabetes and Metabolism Case Reports, 2020, 2020 (1)
ISSN
2052-0573
Publisher
BioScientifica
Journal / Book Title
Endocrinology, Diabetes and Metabolism Case Reports
Volume
2020
Issue
1
Copyright Statement
© 2020 The authors. This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License (https://creativecommons.org/licenses/by-nc-nd/4.0/).
Subjects
2020
ACTH
Adrenal
Adrenalectomy
Adult
Alpha-blockers
Amlodipine
Antibiotics
Anticoagulants*
Arthralgia
Beta-blockers
Blood pressure
CT scan
Calcium
Cardiology
Catecholamines (plasma)
Co-trimoxazole*
Cortisol
Cortisol (serum)
Cushing's syndrome
Dexamethasone suppression
Diabetes mellitus type 1
Diarrhoea
Echocardiogram
Glucocorticoids
Glucose (blood)
Haematoxylin and eosin staining
Heart rate
Histopathology
Hyperactivity
Hypercortisolaemia
Hyperglycaemia
Hyperpigmentation
Hypertension
Hypokalaemia
Insight into disease pathogenesis or mechanism of therapy
Insulin
Laparoscopic adrenalectomy
Malaise
Male
March
Metanephrines
Metanephrines (plasma)
Metyrapone
Normetanephrine
Palpitations
Phaeochromocytoma
Phenoxybenzamine
Potassium
Potassium chloride
Prednisolone
Propranolol
Resection of tumour
Tremulousness
United Kingdom
Ventricular hypertrophy
Vitamin D
Vomiting
Weight gain
White
Date Publish Online
2020-03-13