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Nelson Syndrome

Synonyms of Nelson Syndrome

  • ACTH-secreting pituitary adenoma
  • Pituitary Tumor after Adrenalectomy

Disorder Subdivisions

  • No subdivisions found.

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 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.


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.

Related Disorders


Standard Therapies

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 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 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

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

Nelson Syndrome Resources



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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

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

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

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

Crousus GP, Nelson syndrome; eMedicine; August 2004

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

Report last updated: 2007/07/23 00:00:00 GMT+0

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