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Nelson Syndrome Treatment & Management

  • Author: Thomas A Wilson, MD; Chief Editor: Stephen Kemp, MD, PhD  more...
 
Updated: Dec 11, 2015
 

Medical Care

Although Nelson syndrome was previously thought to be primarily due to a pituitary macroadenoma, and medical treatment alone was thought to be rarely curative, recent high-resolution MRI that allows detection of microadenomas and the use of a newer class of drugs have shown promising results for medical treatment alone to be possible in some cases.

Case reports have demonstrated successful remission of Nelson syndrome with cabergoline (dopamine receptor agonist), with decline and normalization of adrenocorticotropin (ACTH) levels and resolution of the pituitary macroadenoma or microadenoma.[9, 10, 11]

Various other drugs that have been used in an attempt to medically control tumor growth, with variable success, include octreotide (a parenterally administered somatostatin analog), cyproheptadine with and without bromocriptine, and sodium valproate. Octreotide or other analogue of somatostatin may be helpful to control ACTH levels, although it is only useful as a means of controlling levels until definitive treatment is undertaken because it does not result in appreciable tumor shrinkage. Because it suppresses other hormones, including insulin, it may result in other problems, such as carbohydrate intolerance. In addition, it may cause sludging within the gall bladder.

Radiotherapy is important in the treatment of patients with Nelson syndrome.[12]  Historically, the irradiated field needed to be large, which led to increased risk of damage to the surrounding brain. The most serious long-term problems have included learning and memory difficulties, radiation-induced visual damage, and, most importantly, the risk of secondary tumors. With the advent of newer means of reducing radiation scatter (eg, linear accelerator [LINAC], gamma radiosurgery[13, 14, 15] ), radiotherapy-associated morbidity has significantly decreased and may be helpful in tumors that are not amenable to surgery.

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Surgical Care

Surgery remains one of the best hopes of cure for patients with Nelson syndrome and is the treatment of choice for large tumors that are producing acute compression of the optic apparatus and other vital structures.

Transsphenoidal surgery by an experienced surgeon is optimal because it offers the lowest risk of hypothalamic injury.[16]

Stereotactic radiosurgery, called gamma knife surgery (GKS), has been successfully performed in secretory pituitary tumors (including Nelson syndrome tumors) that are refractory to other surgical interventions.[13, 14, 15, 17, 18]

Determinants of success include the degree of tumor invasion of brain parenchyma, optic apparatus, cavernous sinuses, dura, and bone and the skill of the surgeon.

The goal of surgery is to remove all macroscopic tumor tissue without compromising vital structures.

In cases of incomplete removal or where invasion is present, adjunctive irradiation reduces that rate of recurrence and improves the prognosis.

Incidence of treatment-associated hypopituitarism is high, particularly with large tumors.

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Consultations

See the list of potential consultations below:

  • Pediatric endocrinologist
  • Pediatric ophthalmologist
  • Pediatric neurosurgeon (with expertise in transsphenoidal and/or gamma knife surgery)
  • Radiation oncologist (stereotactic radiosurgical center)
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Diet

No restrictions on diet are necessary.

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Activity

No activity restrictions are initially necessary, although standard restrictions occur following surgical resection of the tumor (see Glucocorticoid Therapy and Cushing Syndrome).

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Contributor Information and Disclosures
Author

Thomas A Wilson, MD Professor of Clinical Pediatrics, Chief and Program Director, Division of Pediatric Endocrinology, Department of Pediatrics, The School of Medicine at Stony Brook University Medical Center

Thomas A Wilson, MD is a member of the following medical societies: Endocrine Society, Pediatric Endocrine Society, Phi Beta Kappa

Disclosure: Nothing to disclose.

Coauthor(s)

George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London) Professor and Chair, First Department of Pediatrics, Athens University Medical School, Aghia Sophia Children's Hospital, Greece; UNESCO Chair on Adolescent Health Care, University of Athens, Greece

George P Chrousos, MD, FAAP, MACP, MACE, FRCP(London) is a member of the following medical societies: American Academy of Pediatrics, American College of Physicians, American Pediatric Society, American Society for Clinical Investigation, Association of American Physicians, Endocrine Society, Pediatric Endocrine Society, Society for Pediatric Research, American College of Endocrinology

Disclosure: Nothing to disclose.

Antony Lafferty, MB, BCh 

Antony Lafferty, MB, BCh is a member of the following medical societies: Endocrine Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Lynne Lipton Levitsky, MD Chief, Pediatric Endocrine Unit, Massachusetts General Hospital; Associate Professor of Pediatrics, Harvard Medical School

Lynne Lipton Levitsky, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Diabetes Association, American Pediatric Society, Endocrine Society, Pediatric Endocrine Society, Society for Pediatric Research

Disclosure: Received grant/research funds from Eli Lilly for pi; Received grant/research funds from NovoNordisk for pi; Received consulting fee from NovoNordisk for consulting; Partner received consulting fee from Onyx Heart Valve for consulting.

Chief Editor

Stephen Kemp, MD, PhD Former Professor, Department of Pediatrics, Section of Pediatric Endocrinology, University of Arkansas for Medical Sciences College of Medicine, Arkansas Children's Hospital

Stephen Kemp, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, American Pediatric Society, Endocrine Society, Phi Beta Kappa, Southern Medical Association, Southern Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

Angelo P Giardino, MD, MPH, PhD Professor and Section Head, Academic General Pediatrics, Baylor College of Medicine; Senior Vice President and Chief Quality Officer, Texas Children’s Hospital

Angelo P Giardino, MD, MPH, PhD is a member of the following medical societies: Academic Pediatric Association, American Academy of Pediatrics, American Professional Society on the Abuse of Children, Harris County Medical Society, International Society for the Prevention of Child Abuse and Neglect, Ray E Helfer Society

Disclosure: Received grant/research funds from Health Resources and Services Administration (HRSA) Integrated Community Systems for CSHCN Grant for other; Received advisory board from Baxter Healthcare Corporation for board membership.

Acknowledgements

Vardhini Desikan, MBBS, MPH Pediatric Endocrinology Fellow, Department of Pediatrics, State University of New York at Stony Brook

Vardhini Desikan, MBBS, MPH is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

References
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  2. Nelson DH, Meakin JW, Dealy JB, et al. ACTH-producing tumor of the pituitary gland. N Engl J Med. 1958 Jul 24. 259(4):161-4. [Medline].

  3. van Aken MO, Pereira AM, van den Berg G, Romijn JA, Veldhuis JD, Roelfsema F. Profound amplification of secretory-burst mass and anomalous regularity of ACTH secretory process in patients with Nelson's syndrome compared with Cushing's disease. Clin Endocrinol (Oxf). 2004 Jun. 60(6):765-72. [Medline].

  4. Chapuis Y, Pitre J, Conti F, et al. Role and operative risk of bilateral adrenalectomy in hypercortisolism. World J Surg. 1996 Sep. 20(7):775-9; discussion 779-80. [Medline].

  5. Tiyadatah BN, Kalavampara SV, Sukumar S, Mathew G, Pooleri GK, Prasanna AT, et al. Bilateral Simultaneous Laparoscopic Adrenalectomy in Cushing's Syndrome: Safe, Effective, and Curative. J Endourol. 2012 Feb. 26(2):157-63. [Medline].

  6. Rizk A, Honegger J, Milian M, Psaras T. Treatment Options in Cushing's Disease. Clin Med Insights Oncol. 2012. 6:75-84. [Medline]. [Full Text].

  7. Biller BM, Grossman AB, Stewart PM, et al. Treatment of adrenocorticotropin-dependent Cushing's syndrome: a consensus statement. J Clin Endocrinol Metab. 2008 Jul. 93(7):2454-62. [Medline].

  8. Barabash R, Moreno-Suárez FG, Rodríguez L, Molina AM, Conejo-Mir J. [Nelson syndrome: a rare cause of generalized hyperpigmentation of the skin]. Actas Dermosifiliogr. 2010 Jan-Feb. 101(1):76-80. [Medline].

  9. Casulari LA, Naves LA, Mello PA, Pereira Neto A, Papadia C. Nelson's syndrome: complete remission with cabergoline but not with bromocriptine or cyproheptadine treatment. Horm Res. 2004. 62(6):300-5. [Medline].

  10. Pivonello R, Faggiano A, Di Salle F, Filippella M, Lombardi G, Colao A. Complete remission of Nelson's syndrome after 1-year treatment with cabergoline. J Endocrinol Invest. 1999 Dec. 22(11):860-5. [Medline].

  11. Shraga-Slutzky I, Shimon I, Weinshtein R. Clinical and biochemical stabilization of Nelson's syndrome with long-term low-dose cabergoline treatment. Pituitary. 2006. 9(2):151-4. [Medline].

  12. Petit JH, Biller BM, Yock TI, et al. Proton stereotactic radiotherapy for persistent adrenocorticotropin-producing adenomas. J Clin Endocrinol Metab. 2008 Feb. 93(2):393-9. [Medline].

  13. Kobayashi T, Kida Y, Mori Y. Gamma knife radiosurgery in the treatment of Cushing disease: long-term results. J Neurosurg. 2002 Dec. 97(5 Suppl):422-8. [Medline].

  14. Wolffenbuttel BH, Kitz K, Beuls EM. Beneficial gamma-knife radiosurgery in a patient with Nelson's syndrome. Clin Neurol Neurosurg. 1998 Mar. 100(1):60-3. [Medline].

  15. Mauermann WJ, Sheehan JP, Chernavvsky DR, Laws ER, Steiner L, Vance ML. Gamma Knife surgery for adrenocorticotropic hormone-producing pituitary adenomas after bilateral adrenalectomy. J Neurosurg. 2007 Jun. 106(6):988-93. [Medline].

  16. Patel J, Eloy JA, Liu JK. Nelson's syndrome: a review of the clinical manifestations, pathophysiology, and treatment strategies. Neurosurg Focus. 2015 Feb. 38 (2):E14. [Medline].

  17. Vik-Mo EO, Oksnes M, Pedersen PH, et al. Gamma knife stereotactic radiosurgery of Nelson syndrome. Eur J Endocrinol. 2009 Feb. 160(2):143-8. [Medline].

  18. Barber TM, Adams E, Wass JA. Nelson syndrome: definition and management. Handb Clin Neurol. 2014. 124:327-37. [Medline].

  19. Azad TD, Veeravagu A, Kumar S, Katznelson L. Nelson Syndrome: Update on Therapeutic Approaches. World Neurosurg. 2015 Jun. 83 (6):1135-40. [Medline].

 
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