Nelson Syndrome Treatment & Management

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

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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