Nelson's Syndrome: An Update

Endocrinol Metab Clin North Am. 2020 Sep;49(3):413-432. doi: 10.1016/j.ecl.2020.05.004.


Nelson's syndrome (NS) is a condition which may develop in patients with Cushing's disease after bilateral adrenalectomy. Although there is no formal consensus on what defines NS, corticotroph tumor growth and/or gradually increasing ACTH levels are important diagnostic elements. Pathogenesis is unclear and well-established predictive factors are lacking; high ACTH during the first year after bilateral adrenalectomy is the most consistently reported predictive parameter. Management is individualized and includes surgery, with or without radiotherapy, radiotherapy alone, and observation; medical treatments have shown inconsistent results. A subset of tumors demonstrates aggressive behavior with challenging management, malignant transformation and poor prognosis.

Keywords: Bilateral adrenalectomy; Corticotroph tumor progression; Cushing’s; Nelson’s syndrome; Tumor growth.

Publication types

  • Review

MeSH terms

  • ACTH-Secreting Pituitary Adenoma / complications
  • ACTH-Secreting Pituitary Adenoma / diagnosis
  • ACTH-Secreting Pituitary Adenoma / epidemiology
  • ACTH-Secreting Pituitary Adenoma / therapy
  • Adenoma / complications
  • Adenoma / diagnosis
  • Adenoma / epidemiology
  • Adenoma / therapy
  • Endocrinology / methods
  • Endocrinology / trends*
  • Humans
  • Medical Oncology / methods
  • Medical Oncology / trends
  • Nelson Syndrome* / diagnosis
  • Nelson Syndrome* / epidemiology
  • Nelson Syndrome* / etiology
  • Nelson Syndrome* / therapy
  • Pituitary ACTH Hypersecretion / diagnosis
  • Pituitary ACTH Hypersecretion / epidemiology
  • Pituitary ACTH Hypersecretion / etiology
  • Pituitary ACTH Hypersecretion / therapy