Key points (List 3 to 5 key points of approximately 25 words each that summarize the main points of the article) 1. Patients with Cushing's disease treated with bilateral adrenalectomy require long-term monitoring for the development of Nelson's syndrome.
SynopsisNelson's syndrome (NS) is a potentially severe condition which may develop in patients with Cushing's disease treated with bilateral adrenalectomy (BAL). Although there is no formal consensus on what defines NS, corticotroph tumor growth and/or gradually increasing ACTH levels are important elements in the diagnostic approach. Pathogenesis of NS is still not clarified and well-established predictive factors for its development are lacking; high ACTH levels during the first year after BAL is the most consistently reported predictive parameter.Management options for NS include surgery combined or not by radiotherapy, radiotherapy alone, and observation, whilst medical treatments have not shown consistent results. Treatment strategies should be individualized. A subset of patients with NS will demonstrate aggressive tumor behavior; predictive factors for this are unknown, management can be challenging and prognosis may be poor with malignant transformation and lethal outcomes..