Nelson Syndrome

Nelson Syndrome

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Nelson Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Pituitary Tumor after Adrenalectomy
  • ACTH-secreting pituitary adenoma

Disorder Subdivisions

  • None

General Discussion

Nelson syndrome is a disorder characterized by abnormal hormone secretion, enlargement of the pituitary gland (hypophysis), and the development of large and invasive growths known as adenomas. It occurs in an estimated 15 to 25 percent of people who undergo surgical removal of the adrenal glands for Cushing disease. Symptoms associated with Nelson syndrome include intense skin discoloration (hyperpigmentation), headaches, vision impairment, and the cessation of menstrual periods in women.

Symptoms

Symptoms of Nelson syndrome include intense skin pigmentation, headaches, visual field disturbances and the cessation of menstrual periods in females. Blood levels of the pituitary hormones adrenocorticotrope hormone (ACTH) and beta-melanocyte stimulating hormone (beta-MSH) are abnormally high. The pituitary gland gets abnormally large in Nelson syndrome, causing headaches and visceral symptoms.

Causes

Nelson syndrome can be caused by surgical removal of the adrenal glands on both sides of the body (bilateral adrenalectomy). Removal of the adrenal glands is a treatment for Cushing disease. Cushing disease is the name given to a condition in which Cushing syndrome, an endocrine disorder, occurs because of the presence of benign (non-cancerous) tumors on the pituitary gland. Following removal of these adrenal glands, some people will develop Nelson syndrome. Growth of a pre-existing or a concealed (occult) tumor of the pituitary gland may also cause this disorder.

Affected Populations

Nelson syndrome affects approximately 15 to 25% of people who have undergone surgical removal of their adrenal glands. It affects males and females in equal numbers. Cases caused by tumors are very rare.

Standard Therapies

Diagnosis

When Nelson syndrome is suspected, blood samples are analyzed for the presence or absence of cortisol and/or aldosterone. Imaging studies such as CAT scans or magnetic resonance imaging may also be used.



Treatment

Treatment for Nelson syndrome consists of radiation to limit abnormal growth of the pituitary gland. If the pituitary gland increases so much in size that it encroaches on surrounding brain structures, it may be surgically removed.



Microsurgical removal of Nelson syndrome adenomas through the bone at the base of the skull (transsphenoidal) may be recommended.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

References

TEXTBOOKS

Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:109.



Berkow R., ed. The Merck Manual-Home Edition.2nd ed. Whitehouse Station, NJ: Merck Research Laboratories; 2003:959.



Wilson JD, Foster DW., eds. Textbook of Endocrinology. 8th ed. W.B. Saunders Company. Philadelphia, PA; 1992:283-84; 286.



JOURNAL ARTICLES

Hentschel SJ, McCutcheon IE. Stereotactic radiosurgery for Cushing disease. Neurosurg Focus. 2004;16:E5.



Jane JA Jr, Vance ML, Woodburn CJ, et al. Stereotactic radiosurgery for hypersecreting pituitary tumors: part of a multimodality approach. Neurosurg Focus. 2003;14:e12.



Kobayashi T, Kida Y, Mori Y. Gamma knife radiosurgery in the treatment of Cushing disease: long-term results. J Neurosurg. 2002;97(5 Suppl):422-28.



Kelly PA, Samandouras G Grossman AB, et al. Neurosurgical treatment of nelson's syndrome. J Clin Endocrinol Metab. 2002;87:5465-69.



Pollock BE, Young WF Jr. Stereotactic radiosurgery for patients with ACTH-producing pituitary adenomas after prior adrenalectomy. Int J Radiat Oncol Biol Phys. 2002;54:640-41.



Xing B, Ren Z, Su C, et al. Microsurgical treatment of Nelson's syndrome. Chin Med J (English). 2002;115:1150-52.



Kasperlik-Zaluska AA, Jeske W. Management of Nelson's syndrome: observations in fifteen patients. Clin Endocrinol (Oxf). 2001;55:819.



Arafah BM, Nasrallah MP. Pituitary tumors, pathophysiology, clinical manifestations and management. Endocr Relat Cancer. 2001;8:287-305



Andreassen M, Kristensen LO: Rosiglitazone for prevention or adjuvant treatment of Nelson's syndrome after bilateral adrenalectomy.;Eur J Endocrinol. 2005 Oct;153(4):503-5



Munir A, Song F, Ince P, Ross R, Newell-Price J: A Pilot Study of Prolonged High Dose Rosiglitazone Therapy (12mg/day) in Nelson's Syndrome. Endocrine Abstracts (2004) 8 OC24



Cushing HW. The basophil adenomas of the pituitary body and their clinical manifestations (pituitary basophilism). Bull Johns Hopkins Hosp 1932;50:137-195.



FROM THE INTERNET

Findling J. What is Nelson's syndrome? Cushing's Support & Research Foundation. nd. 1p.www.csrf.net



Nelson Syndrome. Health On the Net Foundation. Last modified: Wed Mar 9 2005. 3pp.

www.hon.ch/HONselect/RareDiseases/EN/C04.588.609.585.html



What is Nelson's syndrome? Endocrine Surgeon. nd. 1p.

www.endocrinesurgeon.co.uk/adrenals/adrenals9.html



Sidhaye AR. MedlinePlus. Medical Encyclopedia. Cushing's disease - exogenous. Update Date: 8/6/2004. 3pp.

www.nlm.nih.gov/medlineplus/ency/article000389.htm



Crousus GP, Nelson syndrome; eMedicine; August 2004 www.emedicine.com/ped/topic1558.htm

Resources

NIH/National Institute of Neurological Disorders and Stroke

P.O. Box 5801

Bethesda, MD 20824

Tel: (301)496-5751

Fax: (301)402-2186

Tel: (800)352-9424

TDD: (301)468-5981

Internet: http://www.ninds.nih.gov/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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