{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,12]],"date-time":"2026-06-12T18:21:07Z","timestamp":1781288467250,"version":"3.54.1"},"reference-count":178,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["FOC"],"published-print":{"date-parts":[[2015,2]]},"abstract":"<jats:p>Nelson's syndrome is a rare clinical manifestation that occurs in 8%\u201347% of patients as a complication of bilateral adrenalectomy, a procedure that is used to control hypercortisolism in patients with Cushing's disease. First described in 1958 by Dr. Don Nelson, the disease has since become associated with a clinical triad of hyperpigmentation, excessive adrenocorticotropin secretion, and a corticotroph adenoma. Even so, for the past several years the diagnostic criteria and management of Nelson's syndrome have been inadequately studied. The primary treatment for Nelson's syndrome is transsphenoidal surgery. Other stand-alone therapies, which in many cases have been used as adjuvant treatments with surgery, include radiotherapy, radiosurgery, and pharmacotherapy. Prophylactic radiotherapy at the time of bilateral adrenalectomy can prevent Nelson's syndrome (protective effect). The most promising pharmacological agents are temozolomide, octreotide, and pasireotide, but these agents are often administered after transsphenoidal surgery. In murine models, rosiglitazone has shown some efficacy, but these results have not yet been found in human studies. In this article, the authors review the clinical manifestations, pathophysiology, diagnostic criteria, and efficacy of multimodal treatment strategies for Nelson's syndrome.<\/jats:p>","DOI":"10.3171\/2014.10.focus14681","type":"journal-article","created":{"date-parts":[[2015,2,1]],"date-time":"2015-02-01T03:40:53Z","timestamp":1422762053000},"page":"E14","source":"Crossref","is-referenced-by-count":42,"title":["Nelson's syndrome: a review of the clinical manifestations, pathophysiology, and treatment strategies"],"prefix":"10.3171","volume":"38","author":[{"given":"Jimmy","family":"Patel","sequence":"first","affiliation":[{"name":"Departments of Neurological Surgery and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean Anderson","family":"Eloy","sequence":"additional","affiliation":[{"name":"Departments of Neurological Surgery and"},{"name":"Otolaryngology\u2013Head and Neck Surgery, and"},{"name":"Center for Skull Base and Pituitary Surgery, Neurological Institute of New Jersey, Rutgers University, New Jersey Medical School, Newark, New Jersey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James K.","family":"Liu","sequence":"additional","affiliation":[{"name":"Departments of Neurological Surgery and"},{"name":"Otolaryngology\u2013Head and Neck Surgery, and"},{"name":"Center for Skull Base and Pituitary Surgery, Neurological Institute of New Jersey, Rutgers University, New Jersey Medical School, Newark, New Jersey"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"b1-focus14681","series-title":"Am J Ther","first-page":"e110","article-title":"Hormonal secretion and quality of life in Nelson syndrome and Cushing disease after long acting repeatable octreotide: a short series and update","volume":"21","author":"Arregger","year":"2014"},{"key":"b2-focus14681","series-title":"Pituitary","first-page":"209","article-title":"The Nelson 's syndrome\u2026 revisited","volume":"7","author":"Assi\u00e9","year":"2004"},{"issue":"3","key":"b3-focus14681","series-title":"Neurosurg Focus","first-page":"E13","article-title":"Nelson syndrome: comprehensive review of pathophysiology, diagnosis, and management","volume":"23","author":"Banasiak","year":"2007"},{"key":"b4-focus14681","series-title":"Eur J Endocrinol","first-page":"495","article-title":"Nelson 's syndrome","volume":"163","author":"Barber","year":"2010"},{"key":"b5-focus14681","series-title":"Clin Endocrinol (Oxf)","first-page":"301","article-title":"Comparison of preoperative and postoperative ACTH concentrations after bilateral adrenalectomy in Cushing's disease","volume":"18","author":"Barnett","year":"1983"},{"key":"b6-focus14681","series-title":"J Neurosurg","first-page":"195","article-title":"Transsphenoidal microsurgical management of Cushing's disease. Report of 100 cases","volume":"59","author":"Boggan","year":"1983"},{"key":"b7-focus14681","series-title":"Clin Endocrinol (Oxf)","first-page":"531","article-title":"Radiosurgery for pituitary adenomas","volume":"61","author":"Brada","year":"2004"},{"key":"b8-focus14681","series-title":"Surgery","first-page":"1127","article-title":"Current status of adrenalectomy for Cushing's disease","volume":"98","author":"Brunicardi","year":"1985"},{"key":"b9-focus14681","series-title":"Eur J Endocrinol","first-page":"707","article-title":"SOM230: a novel somatostatin peptidomimetic with broad somatotropin release inhibiting factor (SRIF) receptor binding and a unique antisecretory profile","volume":"146","author":"Bruns","year":"2002"},{"key":"b10-focus14681","series-title":"N Engl J Med","first-page":"13","article-title":"Ectopic secretion of corticotropin-releasing factor as a cause of Cushing's syndrome. A clinical, morphologic, and biochemical study","volume":"311","author":"Carey","year":"1984"},{"key":"b11-focus14681","series-title":"Horm Res","first-page":"300","article-title":"Nelson 's syndrome: complete remission with cabergoline but not with bromocriptine or cyproheptadine treatment","volume":"62","author":"Casulari","year":"2004"},{"key":"b12-focus14681","series-title":"J Neurosurg","first-page":"967","article-title":"Transsphenoidal microsurgical treatment of Cushing disease: postoperative assessment of surgical efficacy by application of an overnight low-dose dexamethasone suppression test","volume":"98","author":"Chen","year":"2003"},{"key":"b13-focus14681","series-title":"J Endocrinol","first-page":"R19","article-title":"Treatment of Cushing's disease: a mechanistic update","volume":"223","author":"Cuevas-Ramos","year":"2014"},{"key":"b14-focus14681","series-title":"J Clin Invest","first-page":"1892","article-title":"Altered proopiomelanocortin gene expression in adrenocorticotropin-producing nonpituitary tumors. Comparative studies with corticotropic adenomas and normal pituitaries","volume":"76","author":"de Keyzer","year":"1985"},{"key":"b15-focus14681","series-title":"Clin Endocrinol (Oxf)","first-page":"475","article-title":"Over-expression of vasopressin (V3) and corticotrophin-releasing hormone receptor genes in corticotroph tumours","volume":"49","author":"de Keyzer","year":"1998"},{"key":"b16-focus14681","series-title":"J Neurosurg","first-page":"825","article-title":"Surgical management of adrenocorticotropic hormone-secreting macroadenomas: outcome and challenges in patients with Cushing's disease or Nelson 's syndrome","volume":"103","author":"De Tommasi","year":"2005"},{"key":"b17-focus14681","series-title":"J Clin Endocrinol Metab","first-page":"985","article-title":"The evaluation of sodium valproate in the treatment of Nelson 's syndrome","volume":"56","author":"Dornhorst","year":"1983"},{"key":"b18-focus14681","series-title":"Acta Endocrinol Suppl (Copenh)","first-page":"1973","article-title":"ACTH determinations before and after selective removal of pituitary adenomas in Nelson 's syndrome","volume":"173","author":"Espinoza"},{"key":"b19-focus14681","series-title":"World J Surg","first-page":"462","article-title":"Role of bilateral adrenalectomy in Cushing's disease","volume":"18","author":"Favia","year":"1994"},{"key":"b20-focus14681","series-title":"J Clin Endocrinol Metab","first-page":"472","article-title":"Monoclonality of corticotroph macroadenomas in Cushing's disease","volume":"75","author":"Gicquel","year":"1992"},{"key":"b21-focus14681","series-title":"Surgery","first-page":"147","article-title":"Nelson's syndrome after bilateral adrenalectomy for Cushing's disease","volume":"141","author":"Gil-C\u00e1rdenas","year":"2007"},{"issue":"3","key":"b22-focus14681","series-title":"Neurosurg Focus","first-page":"E10","article-title":"Medical management of Cushing disease","volume":"23","author":"Gross","year":"2007"},{"key":"b23-focus14681","series-title":"Indian J Endocrinol Metab","first-page":"1114","article-title":"Nelson 's syndrome presenting as bilateral oculomotor palsy","volume":"17","author":"Gundgurthi","year":"2013"},{"key":"b24-focus14681","series-title":"Surg Neurol","first-page":"316","article-title":"Pituitary Cushing's syndrome and Nelson's syndrome: diagnostic criteria, surgical therapy, and results","volume":"16","author":"Guthrie","year":"1981"},{"key":"b25-focus14681","series-title":"Surgery","first-page":"1064","article-title":"Quality of life after laparoscopic bilateral adrenalectomy for Cushing's disease","volume":"132","author":"Hawn","year":"2002"},{"key":"b26-focus14681","series-title":"Pituitary","first-page":"153","article-title":"Novel pituitary ligands: peroxisome proliferator activating receptor-gamma","volume":"6","author":"Heaney","year":"2003"},{"key":"b27-focus14681","series-title":"J Clin 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syndrome","volume":"26","author":"Kasperlik-Za\u0142uska","year":"2005"},{"key":"b36-focus14681","series-title":"J Clin Endocrinol Metab","first-page":"1803","article-title":"Sustained improvements in plasma ACTH and clinical status in a patient with Nelson's syndrome treated with pasireotide LAR, a multireceptor somatostatin analog","volume":"98","author":"Katznelson","year":"2013"},{"key":"b37-focus14681","series-title":"Postgrad Med J","first-page":"53","article-title":"The effects of octreotide in a patient with Nelson's syndrome","volume":"72","author":"Kelestimur","year":"1996"},{"issue":"3","key":"b38-focus14681","series-title":"Neurosurg Focus","first-page":"E5","article-title":"Transsphenoidal surgery for Cushing's disease: a review of success rates, remission predictors, management of failed surgery, and Nelson's Syndrome","volume":"23","author":"Kelly","year":"2007"},{"key":"b39-focus14681","series-title":"J Clin Endocrinol Metab","first-page":"5465","article-title":"Neurosurgical treatment of Nelson's syndrome","volume":"87","author":"Kelly","year":"2002"},{"key":"b40-focus14681","series-title":"Clin Endocrinol (Oxf)","first-page":"195","article-title":"Long-term treatment of Nelson's syndrome with sodium valproate","volume":"28","author":"Kelly","year":"1988"},{"key":"b41-focus14681","series-title":"Q J Med","first-page":"224","article-title":"Cushing's disease treated by total adrenalectomy: long-term observations of 43 patients","volume":"52","author":"Kelly","year":"1983"},{"key":"b42-focus14681","series-title":"J Clin Endocrinol Metab","first-page":"887","article-title":"Patient's age is a simple predictive factor for the development of Nelson's syndrome after total adrenalectomy for Cushing's disease","volume":"79","author":"Kemink","year":"1994"},{"key":"b43-focus14681","series-title":"Clin Endocrinol (Oxf)","first-page":"45","article-title":"Management of Nelson's syndrome: observations in 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surgery","volume":"23","author":"Liu","year":"2007"},{"key":"b48-focus14681","series-title":"Acta Endocrinol (Copenh)","first-page":"435","article-title":"Size reduction of an ACTH-secreting pituitary macroadenoma in Nelson's syndrome by sodium valproate: effect of withdrawal and re-institution of treatment","volume":"119","author":"Loli","year":"1988"},{"key":"b49-focus14681","series-title":"Acta Neurochir (Wien)","first-page":"3","article-title":"Evaluation of surgically treated Nelson's syndrome","volume":"65","author":"L\u00fcdecke","year":"1982"},{"key":"b50-focus14681","series-title":"Pituitary","article-title":"Gamma knife radiosurgery for Cushing's disease and Nelson's syndrome","author":"Marek","year":"2014"},{"key":"b51-focus14681","series-title":"Clin Endocrinol (Oxf)","first-page":"315","article-title":"Bilateral adrenalectomy: low mortality and morbidity in Cushing's disease","volume":"39","author":"McCance","year":"1993"},{"key":"b52-focus14681","series-title":"Pituitary","first-page":"257","article-title":"Aspects of anterior pituitary growth, with special reference to corticotrophs","volume":"1","author":"McNicol","year":"1999"},{"key":"b53-focus14681","series-title":"J Clin Endocrinol Metab","first-page":"514","article-title":"Acute effects of bromocriptine, cyproheptadine, and valproic acid on plasma adrenocorticotropin secretion in Nelson's syndrome","volume":"82","author":"Mercado-Asis","year":"1997"},{"key":"b54-focus14681","series-title":"Ann Intern Med","first-page":"731","article-title":"Nelson's syndrome: frequency, prognosis, and effect of prior pituitary irradiation","volume":"85","author":"Moore","year":"1976"},{"key":"b55-focus14681","series-title":"Clin Endocrinol (Oxf)","first-page":"91","article-title":"Longitudinal evaluation of adrenocorticotrophin and beta-lipotrophin plasma levels following bilateral adrenalectomy in patients with Cushing's disease","volume":"39","author":"Moreira","year":"1993"},{"key":"b56-focus14681","series-title":"Eur J Endocrinol","first-page":"115","article-title":"Treatment of Nelson's syndrome with temozolomide","volume":"160","author":"Moyes","year":"2009"},{"key":"b57-focus14681","series-title":"Clin Endocrinol (Oxf)","first-page":"519","article-title":"The PPAR-gamma activator rosiglitazone fails to lower plasma ACTH levels in patients with Nelson's syndrome","volume":"64","author":"Mullan","year":"2006"},{"key":"b58-focus14681","series-title":"World J Surg","first-page":"108","article-title":"Long-term results of total adrenalectomy for Cushing's disease","volume":"24","author":"Nagesser","year":"2000"},{"key":"b59-focus14681","series-title":"N Engl J Med","first-page":"161","article-title":"ACTH-producing tumor of the pituitary gland","volume":"259","author":"Nelson","year":"1958"},{"key":"b60-focus14681","series-title":"Ann Intern Med","first-page":"560","article-title":"ACTH-producing pituitary tumors following adrenalectomy for Cushing's syndrome","volume":"52","author":"Nelson","year":"1960"},{"key":"b61-focus14681","series-title":"J Clin Endocrinol Metab","first-page":"722","article-title":"Plasma ACTH determinations in 58 patients before or after adrenalectomy for Cushing's syndrome","volume":"26","author":"Nelson","year":"1966"},{"key":"b62-focus14681","series-title":"J Clin Endocrinol Metab","first-page":"1020","article-title":"Corticotropin-releasing hormone (CRH) stimulation in Nelson's syndrome: response of adrenocorticotropin secretion to pulse injection and continuous infusion of CRH","volume":"62","author":"Oldfield","year":"1986"},{"issue":"Suppl 1","key":"b63-focus14681","series-title":"Clinics (Sao Paulo)","first-page":"119","article-title":"Temozolomide in aggressive pituitary adenomas and carcinomas","volume":"67","author":"Ortiz","year":"2012"},{"key":"b64-focus14681","series-title":"Int J 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cells","volume":"55","author":"Reseti\u0107","year":"1990"},{"key":"b72-focus14681","series-title":"Pituitary","first-page":"71","article-title":"Clinical factors involved in the recurrence of pituitary adenomas after surgical remission: a structured review and meta-analysis","volume":"15","author":"Roelfsema","year":"2012"},{"issue":"Suppl 1","key":"b73-focus14681","series-title":"Neuroendocrinology","first-page":"47","article-title":"Functional activity of the multi-ligand analog SOM230 at human recombinant somatostatin receptor subtypes supports its usefulness in neuroendocrine tumors","volume":"80","author":"Schmid","year":"2004"},{"key":"b74-focus14681","series-title":"J Clin Endocrinol Metab","first-page":"872","article-title":"Effects of various neuropeptides on the secretion of proopiomelanocortin-derived peptides by a cultured pituitary adenoma causing Nelson's syndrome","volume":"55","author":"Shibasaki","year":"1982"},{"key":"b75-focus14681","series-title":"J Clin Endocrinol 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radiation therapy","volume":"12","author":"Vance","year":"2009"},{"key":"b84-focus14681","series-title":"Eur J Endocrinol","first-page":"143","article-title":"Gamma knife stereotactic radiosurgery of Nelson syndrome","volume":"160","author":"Vik-Mo","year":"2009"},{"key":"b85-focus14681","series-title":"J Neurosurg","first-page":"976","article-title":"Editorial. Gamma Knife surgery for Cushing disease","volume":"106","author":"Weiss","year":"2007"},{"key":"b86-focus14681","series-title":"Clin Endocrinol (Oxf)","first-page":"193","article-title":"The effect of cyproheptadine and\/or bromocriptine on plasma ACTH levels in patients cured of Cushing's disease by bilateral adrenalectomy","volume":"32","author":"Whitehead","year":"1990"},{"key":"b87-focus14681","series-title":"J Clin Neurosci","first-page":"100","article-title":"Cushing's disease: a single centre's experience using the linear accelerator (LINAC) for stereotactic radiosurgery and fractionated stereotactic radiotherapy","volume":"21","author":"Wilson","year":"2014"},{"key":"b88-focus14681","series-title":"Clin Neurol Neurosurg","first-page":"60","article-title":"Beneficial gammaknife radiosurgery in a patient with Nelson's syndrome","volume":"100","author":"Wolffenbuttel","year":"1998"},{"key":"b89-focus14681","series-title":"Endocrinology","first-page":"1653","article-title":"Regulation of corticotropin-releasing factor (CRF) receptors in the rat pituitary gland: effects of adrenalectomy on CRF receptors and corticotroph responses","volume":"116","author":"Wynn","year":"1985"},{"key":"b1-focus14681","series-title":"Am J Ther","first-page":"e110","article-title":"Hormonal secretion and quality of life in Nelson syndrome and Cushing disease after long acting repeatable octreotide: a short series and update","volume":"21","author":"Arregger","year":"2014"},{"key":"b2-focus14681","series-title":"Pituitary","first-page":"209","article-title":"The Nelson 's syndrome\u2026 revisited","volume":"7","author":"Assi\u00e9","year":"2004"},{"issue":"3","key":"b3-focus14681","series-title":"Neurosurg Focus","first-page":"E13","article-title":"Nelson syndrome: comprehensive review of pathophysiology, diagnosis, and management","volume":"23","author":"Banasiak","year":"2007"},{"key":"b4-focus14681","series-title":"Eur J Endocrinol","first-page":"495","article-title":"Nelson 's syndrome","volume":"163","author":"Barber","year":"2010"},{"key":"b5-focus14681","series-title":"Clin Endocrinol (Oxf)","first-page":"301","article-title":"Comparison of preoperative and postoperative ACTH concentrations after bilateral adrenalectomy in Cushing's disease","volume":"18","author":"Barnett","year":"1983"},{"key":"b6-focus14681","series-title":"J Neurosurg","first-page":"195","article-title":"Transsphenoidal microsurgical management of Cushing's disease. Report of 100 cases","volume":"59","author":"Boggan","year":"1983"},{"key":"b7-focus14681","series-title":"Clin Endocrinol (Oxf)","first-page":"531","article-title":"Radiosurgery for pituitary adenomas","volume":"61","author":"Brada","year":"2004"},{"key":"b8-focus14681","series-title":"Surgery","first-page":"1127","article-title":"Current status of adrenalectomy for Cushing's disease","volume":"98","author":"Brunicardi","year":"1985"},{"key":"b9-focus14681","series-title":"Eur J Endocrinol","first-page":"707","article-title":"SOM230: a novel somatostatin peptidomimetic with broad somatotropin release inhibiting factor (SRIF) receptor binding and a unique antisecretory profile","volume":"146","author":"Bruns","year":"2002"},{"key":"b10-focus14681","series-title":"N Engl J Med","first-page":"13","article-title":"Ectopic secretion of corticotropin-releasing factor as a cause of Cushing's syndrome. A clinical, morphologic, and biochemical study","volume":"311","author":"Carey","year":"1984"},{"key":"b11-focus14681","series-title":"Horm Res","first-page":"300","article-title":"Nelson 's syndrome: complete remission with cabergoline but not with bromocriptine or cyproheptadine treatment","volume":"62","author":"Casulari","year":"2004"},{"key":"b12-focus14681","series-title":"J Neurosurg","first-page":"967","article-title":"Transsphenoidal microsurgical treatment of Cushing disease: postoperative assessment of surgical efficacy by application of an overnight low-dose dexamethasone suppression test","volume":"98","author":"Chen","year":"2003"},{"key":"b13-focus14681","series-title":"J Endocrinol","first-page":"R19","article-title":"Treatment of Cushing's disease: a mechanistic update","volume":"223","author":"Cuevas-Ramos","year":"2014"},{"key":"b14-focus14681","series-title":"J Clin Invest","first-page":"1892","article-title":"Altered proopiomelanocortin gene expression in adrenocorticotropin-producing nonpituitary tumors. 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