Endocrine Abstracts

Cyclic Cushing’s syndrome is a rare, probably under-reported disorder, characterized by repeated episodes of cortisol excess interspersed by periods of normal cortisol secretion, due to both ACTH-dependent and independent causes, and should be discerned from mild or subclinical Cushing’s syndrome and pseudo-Cushing’s states.

We present a female patient 52 years old, with cyclic hypercortisolism clinically manifested and biologically confirmed at the age 32 (1994). After a first mild episode, next evaluation identified total remission, taking into discussion a pseudo-Cushing. A new hypercortisolism episode with response to stimulation and inhibition tests pleaded for cyclic Cushing’s disease, but only in 2001 CT revealed a pituitary microadenoma. Technical difficulties made impossible the adenomectomy. In spite of the mild episodic hypercortisolism, the evolution was encumbered by complications–hypertension, diabetes, osteoporosis. Treatment with enzymatic inhibitor (ketoconazole) was started with good initial response, followed by digestive intolerance. The nodular hyperplasia of the left adrenal recommended surgical intervention (laparoscopic left adrenalectomy) with a short remission period followed by new hypercortisolism episodes, more severe and more frequent. A treatment with Dostinex induced a relative remission for a short period. Pituitary surgery and totalisation of adrenalectomy were proposed, but the patient refused. The evolutivity of the disease imposed a more targeted treatment and we included the patient into a multinational study with Pasireotide. After a short period of biological improvement of the disease, the patient presented digestive intolerance and retrieved the consent to continue the study.

Conclusions: Cyclic hypercortisolism can occur regularly or irregularly with intercyclic phases ranging from days to years and the treatment is dependent on its underlying cause. This case is important because of the prolonged evolution, and emphasize the therapeutic difficulties, since our patient had a good initial response to different classes of drugs, afterwards the patient developed resistance and/or intolerance to them.