Nelsonov sindrom nakon bilateralne adrenalektomije

Nelson's syndrome (NS) is a rare but serious condition that may develop in patients with Cushing’s disease following bilateral adrenalectomy (BLA), a surgical intervention employed to control hypercortisolism. NS is characterized by the progressive enlargement of adrenocorticotropic hormone (ACTH)-secreting pituitary adenomas, leading to markedly elevated circulating ACTH levels, diffuse skin hyperpigmentation, and neurological deficits secondary to local mass effects. The pathogenesis of NS primarily involves the removal of cortisol-mediated negative feedback on the hypothalamic-pituitary-adrenal (HPA) axis, which promotes corticotroph tumor proliferation and unchecked ACTH hypersecretion. Genetic mutations, notably in USP8 and BRAF, have been implicated in potentiating tumor aggressiveness. Recent advances in diagnostic practices emphasize dynamic contrast-enhanced magnetic resonance imaging (MRI) and sensitive biochemical assays, which have increasingly supplanted reliance on overt clinical features alone. Therapeutic modalities encompass transsphenoidal surgical resection, radiation therapy, and pharmacological agents such as pasireotide, a somatostatin receptor analogue. Novel therapies like temozolomide, inhibitors of the PI3K/AKT/mTOR signaling axis, and immune checkpoint inhibitors are currently under investigation for refractory or rapidly progressing cases. Nonetheless, management challenges persist, particularly regarding tumor recurrence, treatment resistance, and long-term complications, including hypopituitarism and infection-related morbidity, all of which significantly impair the quality of life. This review integrates contemporary understanding of NS, with emphasis on its molecular pathophysiology, evolving diagnostic landscape, clinical spectrum, and emerging treatment strategies. It further highlights the prophylactic utility of pituitary radiotherapy and the importance of vigilant ACTH surveillance post-BLA. Optimal outcomes necessitate a multidisciplinary, individualized approach focused on early detection, targeted interventions, and continuous follow