Nelson Syndrome

A syndrome characterized by HYPERPIGMENTATION, enlarging pituitary mass, visual defects secondary to compression of the OPTIC CHIASM, and elevated serum ACTH. It is caused by the expansion of an underlying ACTH-SECRETING PITUITARY ADENOMA that grows in the absence of feedback inhibition by adrenal CORTICOSTEROIDS, usually after ADRENALECTOMY.

Networked: 27 relevant articles (2 outcomes, 1 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Pituitary Neoplasms (Pituitary Adenoma)
3.	Adenoma (Adenomas)
4.	Prolactinoma (Prolactinomas)
5.	Cushing Syndrome

Experts

1.	Scheithauer, Bernd W: 2 articles (12/2011 - 12/2010)
2.	Kovacs, Kalman: 2 articles (12/2011 - 12/2010)
3.	Syro, Luis V: 2 articles (12/2011 - 12/2010)
4.	Sanchez, Rocío: 1 article (07/2014)
5.	Arregger, Alejandro L: 1 article (07/2014)
6.	Cardoso, Estela M L: 1 article (07/2014)
7.	Monardes Tumilasci, Elida G: 1 article (07/2014)
8.	Sandoval, Olga B: 1 article (07/2014)
9.	Contreras, Liliana N: 1 article (07/2014)
10.	Barber, T M: 1 article (01/2014)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Nelson Syndrome:

1.	Adrenocorticotropic Hormone (ACTH)FDA Link 01/01/2014 - "Although early cases of Nelson syndrome often presented with the clinical features of large sellar masses, the modern face of Nelson syndrome has changed primarily due to earlier detection (with highly resolved magnetic resonance imaging (MRI) and sensitive ACTH assays) and greater awareness of the condition, resulting in reduced morbidity and mortality. " 07/01/2010 - "Of the 34 (79%) patients available for follow-up, 22 (65%) had adrenocorticotropic hormone levels > 200 ng/ml and 6 (27%) had clinical Nelson syndrome. " 04/22/1974 - "Dissociation of plasma and spinal fluid ACTH in Nelson syndrome." 05/01/1998 - "No patient had elevated ACTH levels, and following curative bilateral adrenalectomy, no patient subsequently developed Nelson syndrome, with follow-up ranging from 1 to 8.5 years. " 12/01/2010 - "Low immunohistochemical expression of MGMT in ACTH secreting pituitary tumors of patients with Nelson syndrome."
2.	Dopamine Agonists (Dopamine Agonist)IBA 12/01/2011 - "In decreasing order, lower scores were encountered in ACTH adenomas in Cushing disease, silent subtype 3 adenomas, untreated GH adenomas, silent corticotroph adenomas of subtypes 1 and 2, dopamine agonist-treated PRL adenomas, ACTH adenomas in Nelson syndrome, and gonadotroph adenomas. " 01/01/1991 - "We present seven cases to illustrate 1) the classic disorder cured by pituitary adenomectomy; 2) persistence of the disease after adenomectomy; 3) Cushing's disease manifesting in the puerperium and remitting with dopamine agonist therapy; 4) a patient whose disease relapsed at least five times during 20 years of treatment by adrenalectomy, pituitary radiation, mitotane, and pituitary adenomectomy; 5) the Nelson syndrome; 6) the ectopic adrenocorticotropic hormone (ACTH) syndrome in a patient with dexamethasone suppressible urinary cortisol who had a pituitary adenoma which stained positively for ACTH but who was not cured by total hypophysectomy; and 7) a patient whose ACTH-secreting tumor proved fatal despite repeated surgical, radiologic and pharmacologic measures."
3.	Hydrocortisone (Cortisol)FDA LinkGeneric 01/01/2014 - "Current hypotheses include the "released negative feedback" mechansism (residual pituitary corticotropinoma cells are "released" from the negative feedback effects of cortisol following TBA), and the "aggressive corticotropinoma" mechanism (Nelson syndrome is most likely to develop in those patients with refractory treatments - including TBA - for an underlying aggressive corticotropinoma). " 01/01/1991 - "We present seven cases to illustrate 1) the classic disorder cured by pituitary adenomectomy; 2) persistence of the disease after adenomectomy; 3) Cushing's disease manifesting in the puerperium and remitting with dopamine agonist therapy; 4) a patient whose disease relapsed at least five times during 20 years of treatment by adrenalectomy, pituitary radiation, mitotane, and pituitary adenomectomy; 5) the Nelson syndrome; 6) the ectopic adrenocorticotropic hormone (ACTH) syndrome in a patient with dexamethasone suppressible urinary cortisol who had a pituitary adenoma which stained positively for ACTH but who was not cured by total hypophysectomy; and 7) a patient whose ACTH-secreting tumor proved fatal despite repeated surgical, radiologic and pharmacologic measures."
4.	Mitotane (Lysodren)FDA Link 01/01/1991 - "We present seven cases to illustrate 1) the classic disorder cured by pituitary adenomectomy; 2) persistence of the disease after adenomectomy; 3) Cushing's disease manifesting in the puerperium and remitting with dopamine agonist therapy; 4) a patient whose disease relapsed at least five times during 20 years of treatment by adrenalectomy, pituitary radiation, mitotane, and pituitary adenomectomy; 5) the Nelson syndrome; 6) the ectopic adrenocorticotropic hormone (ACTH) syndrome in a patient with dexamethasone suppressible urinary cortisol who had a pituitary adenoma which stained positively for ACTH but who was not cured by total hypophysectomy; and 7) a patient whose ACTH-secreting tumor proved fatal despite repeated surgical, radiologic and pharmacologic measures."
5.	Dexamethasone (Maxidex)FDA LinkGeneric 01/01/1991 - "We present seven cases to illustrate 1) the classic disorder cured by pituitary adenomectomy; 2) persistence of the disease after adenomectomy; 3) Cushing's disease manifesting in the puerperium and remitting with dopamine agonist therapy; 4) a patient whose disease relapsed at least five times during 20 years of treatment by adrenalectomy, pituitary radiation, mitotane, and pituitary adenomectomy; 5) the Nelson syndrome; 6) the ectopic adrenocorticotropic hormone (ACTH) syndrome in a patient with dexamethasone suppressible urinary cortisol who had a pituitary adenoma which stained positively for ACTH but who was not cured by total hypophysectomy; and 7) a patient whose ACTH-secreting tumor proved fatal despite repeated surgical, radiologic and pharmacologic measures."
6.	Growth Hormone (Somatotropin)IBA 02/01/1995 - "Growth arrest was common with all types except growth hormone (GH)-releasing adenomas; menstrual irregularities were common with all but adenomas causing Nelson syndrome. " 02/01/2013 - "During the time period of this study, 3004 patients were surgically treated; there were 510 prolactinomas, 822 growth hormone adenomas, 62 adrenocorticotropic hormone-producing adenomas, 8 tumors that produced Nelson syndrome, and 1562 adenomas that were not biologically active. "
7.	HormonesIBA 05/01/2011 - "Adrenalectomized patients require lifelong adrenal hormone replacement and are at risk of Nelson syndrome. " 07/01/2014 - "Clinical management of persistent adrenocorticotropin hormone (ACTH) excess in Nelson syndrome (NS) and Cushing disease (CD) remains a challenge. "
8.	temozolomide (Temodar)FDA LinkGeneric 01/01/2014 - "Other therapies (such as Gamma Knife surgery and temozolomide) play an important role and merit further research into their efficacy and placement in the management pathway of Nelson syndrome. " 12/01/2010 - "Our findings suggest that temozolomide therapy may be of potential use in patients with Nelson syndrome, as these tumors express absent/low levels of MGMT. "
9.	Octreotide (Sandostatin)FDA LinkGeneric 07/01/2014 - "Hormonal secretion and quality of life in Nelson syndrome and Cushing disease after long acting repeatable octreotide: a short series and update."
10.	Testosterone (Sustanon)FDA Link 01/01/1996 - "Excessive testosterone production in a patient with Nelson syndrome and bilateral testicular tumors." 01/01/1996 - "We report a rare case of excessive testosterone production by bilateral testicular tumors in a patient with Nelson syndrome (ACTH-secreting pituitary adenoma after bilateral adrenalectomy in patients with Cushing's disease). "

Therapies and Procedures

1.	Adrenalectomy 01/01/2014 - "Nelson syndrome is an important complication of treatment with total bilateral adrenalectomy (TBA) for patients with refractory Cushing's disease. " 03/28/1998 - "[Nelson-Syndrome: 20 years after bilateral adrenalectomy for Cushing's disease]." 02/01/1992 - "The hypophyses of 13 patients with Nelson syndrome following bilateral adrenalectomy were examined by MRI and CT. " 01/01/1986 - "[Course of 2 pregnancies and labor after bilateral adrenalectomy and Nelson syndrome]." 05/01/1984 - "The four patients underwent curative bilateral total adrenalectomy and did not manifest the Nelson syndrome postoperatively (follow-up, 2-22 years). "
2.	Therapeutics 12/01/2010 - "Few medical therapies are available for Nelson syndrome, and the efficacy of such therapeutics remains limited. "
3.	Hypophysectomy 01/01/1991 - "We present seven cases to illustrate 1) the classic disorder cured by pituitary adenomectomy; 2) persistence of the disease after adenomectomy; 3) Cushing's disease manifesting in the puerperium and remitting with dopamine agonist therapy; 4) a patient whose disease relapsed at least five times during 20 years of treatment by adrenalectomy, pituitary radiation, mitotane, and pituitary adenomectomy; 5) the Nelson syndrome; 6) the ectopic adrenocorticotropic hormone (ACTH) syndrome in a patient with dexamethasone suppressible urinary cortisol who had a pituitary adenoma which stained positively for ACTH but who was not cured by total hypophysectomy; and 7) a patient whose ACTH-secreting tumor proved fatal despite repeated surgical, radiologic and pharmacologic measures."
4.	Radiotherapy 01/01/2014 - "Effective management of Nelson syndrome with pituitary surgery and radiotherapy is often a challenge. " 01/01/2014 - "Although lack of administration of neoadjuvant pituitary radiotherapy post-TBA surgery may predict future development of Nelson syndrome, other predictive factors remain controversial. " 04/01/2008 - "After a complete work-up, he was diagnosed with pituitary Cushing syndrome and treated with a bilateral adrenalectomy for Cushing syndrome and pituitary radiotherapy for Nelson syndrome. "