Unusual causes of Cushing's syndrome.
Although in the majority of the patients with Cushing's syndrome (CS), hypercortisolism is due to ACTH hypersecretion by a pituitary tumour or to ectopic ACTH secretion from an extrapituitary neoplastic lesion or to autonomous cortisol secretion by an adrenal tumour, in occasional patients a much rarer entity may be the cause of the syndrome. Herein, we attempted to summarise and categorise these unusual causes according to their presumed aetiology. To this end, we performed a comprehensive computer-based search for unusual or rare causes of CS. The following unusual forms of CS were identified: (i) ACTH hyperesecretion due to ectopic corticotroph adenomas in the parasellar region or the neurohypophysis, or as part of double adenomas, or gangliocytomas; (ii) ACTH hypersecretion due to ectopic CRH or CRH-like peptide secretion by various neoplasms; (iii) ACTH-independent cortisol hypersecretion from ectopic or bilateral adrenal adenomas; (iv) glucocorticoid hypersensitivity; (v) iatrogenic, due to megestrol administration or to ritonavir and fluticasone co-administration. Such unusual presentations of CS illustrate why Cushing's syndrome represents one of the most puzzling endocrine syndromes.
Vassiliadi
Dimitra
D
Department of Endocrinology, Division of Medical Sciences, Institute of Biomedical Research, University of Birmingham, Birmingham, UK.
Tsagarakis
Stylianos
S
D000182:ACTH Syndrome, Ectopic / Q000150:complications; D000310:Adrenal Gland Neoplasms / Q000150:complications; D003480:Cushing Syndrome / Q000209:etiology*; D005260:Female; D005938:Glucocorticoids / Q000009:adverse effects; D006801:Humans; D008113:Liver Neoplasms / Q000150:complications; D019290:Megestrol Acetate / Q000009:adverse effects; D010051:Ovarian Neoplasms / Q000150:complications; D010911:Pituitary Neoplasms / Q000150:complications; D019438:Ritonavir / Q000009:adverse effects
D005938:Glucocorticoids; D019438:Ritonavir; D019290:Megestrol Acetate