Nelson’s syndrome: an update

Athanasios Fountas, Niki Karavitaki

Research output: Contribution to journalArticlepeer-review

2 Citations (Scopus)
200 Downloads (Pure)

Abstract

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.

Original languageEnglish
Pages (from-to)413-432
Number of pages20
JournalEndocrinology and Metabolism Clinics of North America
Volume49
Issue number3
Early online date30 Jul 2020
DOIs
Publication statusPublished - Sept 2020

Keywords

  • Bilateral adrenalectomy
  • Corticotroph tumor progression
  • Cushing's
  • Nelson's syndrome
  • Tumor growth

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Endocrinology

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