Nelson's Syndrome is a complication of removal of the adrenal gland (for the treatment of Cushing's syndrome) when there is a pituitary tumor secreting ACTH (Cushing's disease).
This clinical condition results when a patient with an ACTH-producing pituitary adenoma undergoes bilateral adrenal gland removal (adrenalectomy). Approximately 15-25% of patients with Cushing's disease treated with adrenalectomy will develop Nelson's syndrome within 1 to 4 years. Because these ACTH-secreting tumors are typically aggressive and invasive in their growth, patients may develop large tumors resulting in visual loss, pituitary failure (hypopituitarism) and headache. These adenomas also secrete melanocyte-stimulating hormone, resulting in characteristic hyperpigmentation of the skin. ACTH blood levels are generally markedly elevated but hypercortisolemia is absent.
Transsphenoidal surgery is generally the primary therapy, however cure is often difficult. Radiotherapy, preferably with stereotactic radiation, is effective in controlling tumor growth in the majority of patients who have residual tumor after surgery.
Nelson's on the Message Boards
* Nelson’s syndrome is an iatrogenic condition (caused by a physician) that occurs as a result of treating Cushing's disease patients by removal of both adrenal glands.
* In Cushing’s disease, blood cortisol is elevated due to excessive secretion of the hormone ACTH by a pituitary adenoma. ACTH signals the adrenal gland to produce cortisol. In the past, the identification of a pituitary adenoma was more difficult, and therefore patients underwent removal of the adrenal glands in order to lower the blood levels of cortisol. Because the pituitary tumor was not removed, the tumor enlarged and excessive production of ACTH continued. The hormone ACTH, in excessive amounts, can stimulate pigment production in the skin. Nelson’ syndrome develops within one to four years of surgery in about 15 percent to 25 percent of patients.
* A key finding is darkening of skin color and increased pigmentation of nevi.
* Because ACTH-secreting tumors are typically aggressive and invasive, patients may develop large tumors resulting in visual loss, pituitary failure and headache.
* The clinical history and skin pigmentation findings are usually obvious.
* Hormonal tests generally show elevated ACTH levels in the blood but low or normal levels of cortisol.
* Magnetic Resonance Imaging (MRI) or computed tomography (CT) scan is important for the identification of a pituitary adenoma.
* Transsphenoidal surgery is the primary option but not always effective. Radiotherapy, preferably with stereotactic radiation, controls tumor growth in the majority of patients with residual tumor growth after surgery.
The Neuro-ICU cares for patients with all types of neurosurgical and neurological injuries, including stroke, brain hemorrhage, trauma and tumors. We work in close cooperation with your surgeon or medical doctor with whom you have had initial contact. Together with the surgeon or medical doctor, the NeuroICU attending physician and team members direct your family member's care while in the ICU. The NeuroICU team consists of the bedside nurses, nurse practioners, physicians in specialty training (Fellows) and attending physicians.
- ^ Biller BM, Grossman AB, Stewart PM, et al. (July 2008). "Treatment of adrenocorticotropin-dependent Cushing's syndrome: a consensus statement". J. Clin. Endocrinol. Metab. 93 (7): 2454–62. doi:10.1210/jc.2007-2734. PMID 18413427. http://jcem.endojournals.org/cgi/content/full/93/7/2454.
- ^ Kelly PA, Samandouras G, Grossman AB, Afshar F, Besser GM, Jenkins PJ (December 2002). "Neurosurgical treatment of Nelson's syndrome". J. Clin. Endocrinol. Metab. 87 (12): 5465–9. doi:10.1210/jc.2002-020299. PMID 12466338. http://jcem.endojournals.org/cgi/pmidlookup?view=long&pmid=12466338.
- ^ Nelson D, Meakin J, Thorn G (1960). "ACTH-producing pituitary tumors following adrenalectomy for Cushing's syndrome". Ann Intern Med 52: 560–9. PMID 14426442.