Nelson's syndrome represents a rare but possible clinical entity in patients with Cushing's disease treated with total bilateral adrenalectomy. It is usually accompanied by tumor expansion of the corticotropinoma, visual field involvement due to suprasellar extension, and skin pigmentation changes. The biochemical criteria for its diagnosis are not unified, and its pathophysiology is still under study to better understand the molecular mechanisms that give rise to it. Several therapeutic options range from reoperation of the corticotropinoma, use of drugs, and radiotherapy modalities. This article reviews the current points regarding the diagnosis, pathophysiology, and management of Nelson´s syndrome.
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