ABSTRACT
Cushing syndrome (CS) is a rare feature of McCune-Albright syndrome. Treatments consist of bilateral adrenalectomy followed by lifelong glucocorticoid and mineralocorticoid treatment. However, cases of spontaneous remission of CS have been reported in the literature. We report a case of McCune-Albright syndrome with CS treated with metyrapone for 30 months with prolonged remission after a 12-year follow-up. Adrenalectomy may be avoided in some cases of CS caused by McCune-Albright syndrome. Metyrapone could be a good alternative to surgical treatment.
Subject(s)
Cushing Syndrome/drug therapy , Enzyme Inhibitors/therapeutic use , Fibrous Dysplasia, Polyostotic/complications , Metyrapone/therapeutic use , Child , Cushing Syndrome/etiology , Female , Follow-Up Studies , Humans , Remission InductionABSTRACT
UNLABELLED: Cushing's syndrome reveals McCune-Albright syndrome in a minority of patients. Bilateral adrenalectomy is performed on the majority of cases. CASE REPORT: We present a case of McCune-Albright syndrome revealed by a Cushing's syndrome treated by metyrapone. Results were good, after a 4-year follow up. CONCLUSION: Treatment with metyrapone could be recommended, for some patients, in McCune-Albright syndrome with Cushing's syndrome before chirurgical treatment.
