Endocrine Abstracts

Introduction: Cushing’s disease is caused by endogenous hypercotisolism, due to the hypersecretion of the adenocorticotropic hormone (ACTH) by an ACTH-secreting pituitary adenoma. However, some patients with Cushing’s disease have no visible adenoma on MRI. The goal of this study is to review the clinical features, diagnosis and medical preparation of patients with Cushing’s disease.

Material and Methods: A retrospective study including 6 patients with Cushing’s disease and a negative MRI in the Department of Endocrinology-Diabetology of Oujda’s University Hospital.

Results: The mean age of the patients was 33.6 years with a female predominance. All of the patients showed clinical signs of hypercortisolism. Elevated midnight blood cortisol and 24-hour urinary free cortisol as well as non-suppression of cortisol during 1mg overnight dexamethasone suppression test were observed in all cases. 100% of the patients had an elevated ACTH value and responded to 8mg dexamethasone suppression test. The hypothalamic-pituitary MRI was normal in all cases. The decisions considering treatment for each patient were discussed over multidisciplinary meetings. Thus, all of the patients have undergone preoperative medical therapies: 50% of the patients were treated with Metopirone, and 50% with Ketoconazole. All patients had a bilateral adrenalectomy, with a successful recovery from hypercortisolism. No case of Nelson’s syndrome has been registered thus far.

Discussion and conclusion

Inferior petrosal sinus sampling (IPSS), though being invasive, is the gold standard for establishing the pituitary origin of ACTH secretion in patients with negative MRI and help guide the neurosurgical exploration using the endonasal transsphenoidal approach. However, in developping countries such as ours, this option is not available. Bilateral adrenalectomy seems more reliable and efficient in our context, but requires lifelong glucocorticoid and mineralocorticoid replacement with an increased risk of Nelson’s syndrome.