Nelson Syndrome: Update on Therapeutic Approaches

Abstract

Objective: To review the pathophysiology and therapeutic modalities availble for Nelson syndrome.

Methods: We reviewed the current literature including managment for Nelson syndrome.

Results: For patients with NS, surgical intervention is often the first-line therapy. With refractory NS or tumors with extrasellar involvement, radiosurgery offers an important alternative or adjuvant option. Pharmacologic interventions have demonstrated limited usefulness, although recent evidence supports the feasibility of a novel somatostatin analog for patients with NS. Modern neuroimaging, improved surgical techniques, and the advent of stereotactic radiotherapy have transformed the management of NS.

Conclusions: An up-to-date understanding of the pathophysiology underlying Nelson Syndrome and evidence-based management is imperative. Early detection may allow for more successful therapy in patients with Nelson Syndrome. Improved radiotherapeutic interventions and rapidly evolving pharmacologic therapies offer an opportunity to create targeted, multifocal treatment regiments for patients with Nelson Syndrome.

Keywords: ACTH; Bilateral adrenalectomy; Cushing's disease; Nelson syndrome; Pituitary.