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 language | English |
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Pages (from-to) | 413-432 |
Number of pages | 20 |
Journal | Endocrinology and Metabolism Clinics of North America |
Volume | 49 |
Issue number | 3 |
Early online date | 30 Jul 2020 |
DOIs | |
Publication status | Published - Sept 2020 |
Keywords
- Bilateral adrenalectomy
- Corticotroph tumor progression
- Cushing's
- Nelson's syndrome
- Tumor growth
ASJC Scopus subject areas
- Endocrinology, Diabetes and Metabolism
- Endocrinology