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Nelson Syndrome

MedGen UID:
6544
•Concept ID:
C0027577
•
Neoplastic Process
Synonym: Nelson syndrome
SNOMED CT: Nelson syndrome (43019009)

Definition

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. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNelson Syndrome
Follow this link to review classifications for Nelson Syndrome in Orphanet.

Professional guidelines

PubMed

Radiotherapy as a tool for the treatment of Cushing's disease.
Castinetti F, Brue T, Ragnarsson O
Eur J Endocrinol 2019 May 1;180(5):D9-D18. doi: 10.1530/EJE-19-0092. PMID: 30970325
Nelson syndrome: definition and management.
Barber TM, Adams E, Wass JA
Handb Clin Neurol 2014;124:327-37. doi: 10.1016/B978-0-444-59602-4.00022-8. PMID: 25248597
Nelson syndrome: comprehensive review of pathophysiology, diagnosis, and management.
Banasiak MJ, Malek AR
Neurosurg Focus 2007;23(3):E13. doi: 10.3171/foc.2007.23.3.15. PMID: 17961028

Recent clinical studies

Etiology

Updates in the outcomes of radiation therapy for Cushing's disease.
Gheorghiu ML
Best Pract Res Clin Endocrinol Metab 2021 Mar;35(2):101514. Epub 2021 Mar 10 doi: 10.1016/j.beem.2021.101514. PMID: 33814300
Re-examining Nelson's syndrome.
Palermo NE, Ananthakrishnan S
Curr Opin Endocrinol Diabetes Obes 2015 Aug;22(4):313-8. doi: 10.1097/MED.0000000000000175. PMID: 26087343
Management of Cushing disease.
Tritos NA, Biller BM, Swearingen B
Nat Rev Endocrinol 2011 May;7(5):279-89. Epub 2011 Feb 8 doi: 10.1038/nrendo.2011.12. PMID: 21301487
"Silent"corticotropinoma.
Pawlikowski M, Kunert-Radek J, Radek M
Neuro Endocrinol Lett 2008 Jun;29(3):347-50. PMID: 18580839
Cushing's disease and Nelson's syndrome.
Wilson CB, Tyrrell JB, Fitzgerald PA, Pitts LH
Clin Neurosurg 1980;27:19-30. doi: 10.1093/neurosurgery/27.cn_suppl_1.19. PMID: 7273554

Diagnosis

Management of Nelson's Syndrome.
Fountas A, Karavitaki N
Medicina (Kaunas) 2022 Nov 2;58(11) doi: 10.3390/medicina58111580. PMID: 36363537Free PMC Article
Nelson's Syndrome: An Update.
Fountas A, Karavitaki N
Endocrinol Metab Clin North Am 2020 Sep;49(3):413-432. doi: 10.1016/j.ecl.2020.05.004. PMID: 32741480
Ectopic Adrenocorticotropic Hormone-Secreting Pituitary Adenomas: An Underestimated Entity.
Knappe UJ, Jaspers C, Buschsieweke D, Reinbold WD, Alomari A, Saeger W, Ehlenz K, Mann WA, Kann PH, Feldkamp J
Neurosurgery 2017 Apr 1;80(4):525-533. doi: 10.1227/NEU.0000000000001319. PMID: 27352274
Nelson's Syndrome.
Munir A, Newell-Price J
Arq Bras Endocrinol Metabol 2007 Nov;51(8):1392-6. doi: 10.1590/s0004-27302007000800026. PMID: 18209878
Cushing's disease and Nelson's syndrome.
Wilson CB, Tyrrell JB, Fitzgerald PA, Pitts LH
Clin Neurosurg 1980;27:19-30. doi: 10.1093/neurosurgery/27.cn_suppl_1.19. PMID: 7273554

Therapy

Management of Nelson's Syndrome.
Fountas A, Karavitaki N
Medicina (Kaunas) 2022 Nov 2;58(11) doi: 10.3390/medicina58111580. PMID: 36363537Free PMC Article
Nelson's syndrome: a review of the clinical manifestations, pathophysiology, and treatment strategies.
Patel J, Eloy JA, Liu JK
Neurosurg Focus 2015 Feb;38(2):E14. doi: 10.3171/2014.10.FOCUS14681. PMID: 25639316
Nelson's syndrome.
Barber TM, Adams E, Ansorge O, Byrne JV, Karavitaki N, Wass JA
Eur J Endocrinol 2010 Oct;163(4):495-507. Epub 2010 Jul 28 doi: 10.1530/EJE-10-0466. PMID: 20668020
Nelson's Syndrome.
Munir A, Newell-Price J
Arq Bras Endocrinol Metabol 2007 Nov;51(8):1392-6. doi: 10.1590/s0004-27302007000800026. PMID: 18209878
Somatostatin.
Efendić S, Hökfelt T, Luft R
Adv Metab Disord 1978;9:367-424. doi: 10.1016/b978-0-12-027309-6.50023-9. PMID: 347908

Prognosis

Gamma knife radiosurgery in patients with Nelson's syndrome.
Losa M, Detomas M, Bailo M, Barzaghi LR, Albano L, Piloni M, Pagnano A, Pedone E, Mortini P
J Endocrinol Invest 2021 Oct;44(10):2243-2251. Epub 2021 Feb 20 doi: 10.1007/s40618-021-01531-6. PMID: 33611756
Nelson's Syndrome: An Update.
Fountas A, Karavitaki N
Endocrinol Metab Clin North Am 2020 Sep;49(3):413-432. doi: 10.1016/j.ecl.2020.05.004. PMID: 32741480
Re-examining Nelson's syndrome.
Palermo NE, Ananthakrishnan S
Curr Opin Endocrinol Diabetes Obes 2015 Aug;22(4):313-8. doi: 10.1097/MED.0000000000000175. PMID: 26087343
Nelson's syndrome.
Barber TM, Adams E, Ansorge O, Byrne JV, Karavitaki N, Wass JA
Eur J Endocrinol 2010 Oct;163(4):495-507. Epub 2010 Jul 28 doi: 10.1530/EJE-10-0466. PMID: 20668020
Cushing's disease and Nelson's syndrome.
Wilson CB, Tyrrell JB, Fitzgerald PA, Pitts LH
Clin Neurosurg 1980;27:19-30. doi: 10.1093/neurosurgery/27.cn_suppl_1.19. PMID: 7273554

Clinical prediction guides

Management of Nelson's Syndrome.
Fountas A, Karavitaki N
Medicina (Kaunas) 2022 Nov 2;58(11) doi: 10.3390/medicina58111580. PMID: 36363537Free PMC Article
Gamma knife radiosurgery in patients with Nelson's syndrome.
Losa M, Detomas M, Bailo M, Barzaghi LR, Albano L, Piloni M, Pagnano A, Pedone E, Mortini P
J Endocrinol Invest 2021 Oct;44(10):2243-2251. Epub 2021 Feb 20 doi: 10.1007/s40618-021-01531-6. PMID: 33611756
Nelson's Syndrome: An Update.
Fountas A, Karavitaki N
Endocrinol Metab Clin North Am 2020 Sep;49(3):413-432. doi: 10.1016/j.ecl.2020.05.004. PMID: 32741480
Nelson's syndrome.
Barber TM, Adams E, Ansorge O, Byrne JV, Karavitaki N, Wass JA
Eur J Endocrinol 2010 Oct;163(4):495-507. Epub 2010 Jul 28 doi: 10.1530/EJE-10-0466. PMID: 20668020
"Silent"corticotropinoma.
Pawlikowski M, Kunert-Radek J, Radek M
Neuro Endocrinol Lett 2008 Jun;29(3):347-50. PMID: 18580839

Recent systematic reviews

Nelson syndrome and perinatal challenges: A case report and systematic review of the literature.
Jones K, Reis I, Levy MJ, Potdar N
Int J Gynaecol Obstet 2023 Oct;163(1):89-95. Epub 2023 May 2 doi: 10.1002/ijgo.14791. PMID: 37128819
Prevalence of Nelson's syndrome after bilateral adrenalectomy in patients with cushing's disease: a systematic review and meta-analysis.
Papakokkinou E, Piasecka M, Carlsen HK, Chantzichristos D, Olsson DS, Dahlqvist P, Petersson M, Berinder K, Bensing S, Höybye C, Engström BE, Burman P, Follin C, Petranek D, Erfurth EM, Wahlberg J, Ekman B, Åkerman AK, Schwarcz E, Johannsson G, Falhammar H, Ragnarsson O
Pituitary 2021 Oct;24(5):797-809. Epub 2021 May 25 doi: 10.1007/s11102-021-01158-z. PMID: 34036460Free PMC Article
Corticotroph tumor progression after bilateral adrenalectomy (Nelson's syndrome): systematic review and expert consensus recommendations.
Reincke M, Albani A, Assie G, Bancos I, Brue T, Buchfelder M, Chabre O, Ceccato F, Daniele A, Detomas M, Di Dalmazi G, Elenkova A, Findling J, Grossman AB, Gomez-Sanchez CE, Heaney AP, Honegger J, Karavitaki N, Lacroix A, Laws ER, Losa M, Murakami M, Newell-Price J, Pecori Giraldi F, Pérez-Rivas LG, Pivonello R, Rainey WE, Sbiera S, Schopohl J, Stratakis CA, Theodoropoulou M, van Rossum EFC, Valassi E, Zacharieva S, Rubinstein G, Ritzel K
Eur J Endocrinol 2021 Mar;184(3):P1-P16. doi: 10.1530/EJE-20-1088. PMID: 33444221Free PMC Article

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