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A syndrome that develops following bilateral adrenalectomy for Cushing syndrome. The signs and symptoms result from the presence of an adenocorticotropin-secreting pituitary gland adenoma, and include enlargement of the sella turcica and pressure on the adjacent structures, and skin hyperpigmentation.
Features include always present findings: Secondary hypercortisolism and Adrenocorticotropic hormone excess; and very common findings: Increased circulating cortisol level, Pituitary corticotropic cell adenoma, and Increased urinary cortisol level. 27 total HPO annotations.
Organ System | Phenotype Count | Example Features |
|---|---|---|
Hormones | 6 | Increased circulating cortisol level, Pituitary corticotropic cell adenoma, Increased urinary cortisol level |
Biomarker and diagnostic research for Nelson syndrome has been reported in the published literature.
Phenotype severity distribution: 2 always present features, 3 very common features, 10 common features.
No clinical trials have been registered for Nelson syndrome.
12 publications have been identified in PubMed for Nelson syndrome. Research spans Other (27%), Review / Meta-Analysis (27%), and Case Report / Case Series (27%).
Research Type | Count | % of Total |
|---|---|---|
Other research | 3 | 27% |
Data assembled from 4 of 12 sources · Last updated Sep 19, 2026, 6:43 AM UTC
European rare disease database
Genetic and Rare Diseases Info Center
Common questions about Nelson syndrome
Eyes | 4 | Visual impairment, Optic nerve compression, Oculomotor nerve palsy |
Heart and blood vessels | 2 | Hypertension, Intracranial hemorrhage |
Muscles | 2 | Lower limb muscle weakness, Quadriceps muscle atrophy |
Kidneys and urinary system | 1 | Increased urinary cortisol level |
Arms and legs | 1 | Lower limb muscle weakness |
Skin | 1 | Generalized hyperpigmentation |
Lab test results | 1 | Increased circulating prolactin concentration |
3 |
27% |
Patient case studies | 3 | 27% |
Testing and diagnosis research | 1 | 9% |
Laboratory research | 1 | 9% |
Wang M (2026). [PMID: 42032542](https://pubmed.ncbi.nlm.nih.gov/42032542/). *BMC Endocr Disord*. [Case Report / Case Series]
Monserrate AE (2026). [PMID: 32809590](https://pubmed.ncbi.nlm.nih.gov/32809590/). *Unknown Journal*. [Other]
Cécilia L (2026). [PMID: 41655900](https://pubmed.ncbi.nlm.nih.gov/41655900/). *Ann Endocrinol (Paris)*. [Other]
Javvadi AB (2026). [PMID: 41853041](https://pubmed.ncbi.nlm.nih.gov/41853041/). *J Pharm Bioallied Sci*. [Review / Meta-Analysis]
Hsiao IT (2026). [PMID: 41815596](https://pubmed.ncbi.nlm.nih.gov/41815596/). *Cureus*. [Case Report / Case Series]
Losa M (2025). [PMID: 41359197](https://pubmed.ncbi.nlm.nih.gov/41359197/). *Pituitary*. [Other]
Hajikarimloo B (2025). [PMID: 40884684](https://pubmed.ncbi.nlm.nih.gov/40884684/). *Pituitary*. [Review / Meta-Analysis]
Mazzeo P (2025). [PMID: 41006788](https://pubmed.ncbi.nlm.nih.gov/41006788/). *Pituitary*. [Diagnostic / Biomarker]
Sadhwani N (2024). [PMID: 38734175](https://pubmed.ncbi.nlm.nih.gov/38734175/). *World Neurosurg*. [Basic Science / Preclinical]
Esquivel JE (2024). [PMID: 39099974](https://pubmed.ncbi.nlm.nih.gov/39099974/). *Cureus*. [Case Report / Case Series]