Pregledni rad

https://doi.org/10.57140/mj.55.3-4.4

Nelson’s Syndrome after bilateral adrenalectomy: A comprehensive review of pathogenesis, diagnosis, and treatment approaches

Vinod Kumar Mugada orcid.org/0000-0002-9364-9874 ; Department of Pharmacy Practice, Vignan Institute of Pharmaceutical Technology, Duvvada, AP, India
Ravulapalli Bala Bhavana ; Department of Pharmacy Practice, Vignan Institute of Pharmaceutical Technology, Duvvada, AP, India; Pharm. D Intern, Visakha Institute of Medical Sciences, Hanumanthuwaka, Visakhapatnam, AP, India
Srinivasa Rao Yarguntla ; Department of Pharmaceutics, Vignan Institute of Pharmaceutical Technology, Duvvada, AP, India
Saritha Medapati ; Department of Pharmaceutics, Vignan Institute of Pharmaceutical Technology, Duvvada, AP, India
Nikita Vavalapilli ; Department of Pharmacy Practice, Vignan Institute of Pharmaceutical Technology, Duvvada, AP, India; Pharm. D Intern, Visakha Institute of Medical Sciences, Hanumanthuwaka, Visakhapatnam, AP, India

Sažetak

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-up.

Ključne riječi

Nelson’s syndrome; Cushing’s disease; post-adrenalectomy; ACTH-secreting pituitary adenoma

Hrčak ID:

342752

URI

https://hrcak.srce.hr/342752

Datum izdavanja:

8.1.2026.

Posjeta: 605 *