Atypical Nelson Syndrome Following Right Partial and Left Total Nephrectomy With Incidental Bilateral Total...

Nelson syndrome (NS) is a dangerous condition that can sometimes manifest after bilateral adrenalectomy (BA), typically in treating Cushing's disease. It is defined by the collection of systemic signs and symptoms that can arise in a state where there are chronically and massively elevated levels of adrenocorticotropic hormone (ACTH). Traditionally it may manifest from six months to 24 years following the loss of both adrenal glands, with the meantime of development being 15 years following BA. The diagnostic criteria are controversial, with historically many different methods being used, ranging from visual field defects and an enlarged pituitary corticotrophinoma to elevated plasma ACTH levels and skin hyperpigmentation. What remains consistent between criteria is that it is secondary to total BA, traditionally in treating refractory Cushing's disease. We describe here a rare case of a patient diagnosed with bilateral renal cell carcinoma (RCC) treated with right partial and left total nephrectomy, and incidental BA, presenting with the symptoms and signs of NS. Although NS classically presents following total BA for the treatment of Cushing disease, further research is required to look for etiologies of Nelson's-like pathology outside the context of Cushing's disease treatment, thereby necessitating a change to the traditional diagnostic criteria for the syndrome to identify cases that would otherwise go untreated. In addition, this case report's outlining, drafting, and conclusions were written in part by or with the support of Chat Generative Pre-Trained Transformer (ChatGPT), a large language transformer open-source artificial intelligence.