Gamma knife stereotactic radiosurgery of Nelson syndrome

Vik-Mo, Einar O.; Øksnes, Marianne; Pedersen, Paal‐Henning; Wentzel‐Larsen, Tore; Rødahl, Eyvind; Thorsen, Frits; Schreiner, Thomas; Aanderud, Sylvi; Lund‐Johansen, Morten

doi:10.1530/eje-08-0687

Cited by 40 publications

(25 citation statements)

References 14 publications

(23 reference statements)

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“…7,43,50,85,70 Vik-Mo et al published a study in which 10 patients with Nelson's syndrome underwent GK treatment. 84 On average, tumor volume decreased by 32% for 9 patients but not at all for 1 patient. Of these 10 patients, ACTH levels decreased for 8 (range 2.0-278 pmol/L) and reached normal range for only 1 patient.…”

Section: Surgical Treatmentmentioning

confidence: 87%

Nelson's syndrome: a review of the clinical manifestations, pathophysiology, and treatment strategies

Patel¹,

Eloy

Liu

2015

FOC

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Nelson's syndrome is a rare clinical manifestation that occurs in 8%–47% of patients as a complication of bilateral adrenalectomy, a procedure that is used to control hypercortisolism in patients with Cushing's disease. First described in 1958 by Dr. Don Nelson, the disease has since become associated with a clinical triad of hyperpigmentation, excessive adrenocorticotropin secretion, and a corticotroph adenoma. Even so, for the past several years the diagnostic criteria and management of Nelson's syndrome have been inadequately studied. The primary treatment for Nelson's syndrome is transsphenoidal surgery. Other stand-alone therapies, which in many cases have been used as adjuvant treatments with surgery, include radiotherapy, radiosurgery, and pharmacotherapy. Prophylactic radiotherapy at the time of bilateral adrenalectomy can prevent Nelson's syndrome (protective effect). The most promising pharmacological agents are temozolomide, octreotide, and pasireotide, but these agents are often administered after transsphenoidal surgery. In murine models, rosiglitazone has shown some efficacy, but these results have not yet been found in human studies. In this article, the authors review the clinical manifestations, pathophysiology, diagnostic criteria, and efficacy of multimodal treatment strategies for Nelson's syndrome.

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Section: Surgical Treatmentmentioning

confidence: 87%

Nelson's syndrome: a review of the clinical manifestations, pathophysiology, and treatment strategies

Patel¹,

Eloy

Liu

2015

FOC

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“…CRT has been demonstrated to decrease plasma ACTH levels and induce shrinkage of the pituitary tumor in 93.3% of patients with NS (303). The data on the use of GK, the only type of radiosurgery reported in the management of corticotroph tumor progression, are conflicting, and the remission rate represented by tumor growth arrest and/or decline in circulating ACTH levels, ranges from 14 to 100% (319,(441)(442)(443)(444)(445). Although one study showed tumor growth arrest and no tumor regrowth in 100% of patients at 7 years after GK therapy (444), another study showed remission rate after GK to be only 14% (445).…”

Section: Nelson Syndrome or Corticotroph Tumor Progressionmentioning

confidence: 99%

“…The data on the use of GK, the only type of radiosurgery reported in the management of corticotroph tumor progression, are conflicting, and the remission rate represented by tumor growth arrest and/or decline in circulating ACTH levels, ranges from 14 to 100% (319,(441)(442)(443)(444)(445). Although one study showed tumor growth arrest and no tumor regrowth in 100% of patients at 7 years after GK therapy (444), another study showed remission rate after GK to be only 14% (445). GK seems to be more effective in the treatment of corticotroph tumor progression when administered soon after bilateral adrenalectomy and when the anatomical target is clear and discrete (319,443,444).…”

Section: Nelson Syndrome or Corticotroph Tumor Progressionmentioning

confidence: 99%

The Treatment of Cushing's Disease

et al. 2015

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Cushing's disease (CD), or pituitary-dependent Cushing's syndrome, is a severe endocrine disease caused by a corticotroph pituitary tumor and associated with increased morbidity and mortality. The first-line treatment for CD is pituitary surgery, which is followed by disease remission in around 78% and relapse in around 13% of patients during the 10-year period after surgery, so that nearly one third of patients experience in the long-term a failure of surgery and require an additional second-line treatment. Patients with persistent or recurrent CD require additional treatments, including pituitary radiotherapy, adrenal surgery, and/or medical therapy. Pituitary radiotherapy is effective in controlling cortisol excess in a large percentage of patients, but it is associated with a considerable risk of hypopituitarism. Adrenal surgery is followed by a rapid and definitive control of cortisol excess in nearly all patients, but it induces adrenal insufficiency. Medical therapy has recently acquired a more important role compared to the past, due to the recent employment of novel compounds able to control cortisol secretion or action. Currently, medical therapy is used as a presurgical treatment, particularly for severe disease; or as postsurgical treatment, in cases of failure or incomplete surgical tumor resection; or as bridging therapy before, during, and after radiotherapy while waiting for disease control; or, in selected cases, as primary therapy, mainly when surgery is not an option. The adrenal-directed drug ketoconazole is the most commonly used drug, mainly because of its rapid action, whereas the glucocorticoid receptor antagonist, mifepristone, is highly effective in controlling clinical comorbidities, mainly glucose intolerance, thus being a useful treatment for CD when it is associated with diabetes mellitus. Pituitary-directed drugs have the advantage of acting at the site responsible for CD, the pituitary tumor. Among this group of drugs, the dopamine agonist cabergoline and the somatostatin analog pasireotide result in disease remission in a consistent subgroup of patients with CD. Recently, pasireotide has been approved for the treatment of CD when surgery has failed or when surgery is not an option, and mifepristone has been approved for the treatment of Cushing's syndrome when associated with impairment of glucose metabolism in case of the lack of a surgical indication. Recent experience suggests that the combination of different drugs may be able to control cortisol excess in a great majority of patients with CD.

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“…Although one study of Nelson's syndrome patients showed no tumour re-growth at 7 years post GKS therapy (82), a further study showed remission rates post GKS to be only 14% (83). GKS seems to be most effective in Nelson's syndrome when it is administered soon after TBA (82) and when the anatomical target is clear and discrete (less likely with prior surgery, which may render the tumour border indistinct (78,81)). Potential adverse effects of GKS include panhypopituitarism (up to at least 7 years post surgery (84)) and cranial nerve palsies.…”

Section: Stereotactic Radiosurgerymentioning

confidence: 99%

Nelson's syndrome

Barber

Adams²,

Ansorge³

et al. 2010

European Journal of Endocrinology

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Nelson's syndrome is a potentially life-threatening condition that does not infrequently develop following total bilateral adrenalectomy (TBA) for the treatment of Cushing's disease. In this review article, we discuss some controversial aspects of Nelson's syndrome including diagnosis, predictive factors, aetiology, pathology and management based on data from the existing literature and the experience of our own tertiary centre. Definitive diagnostic criteria for Nelson's syndrome are lacking. We argue in favour of a new set of criteria. We propose that Nelson's syndrome should be diagnosed in any patient with prior TBA for the treatment of Cushing's disease and with at least one of the following criteria: i) an expanding pituitary mass lesion compared with pre-TBA images; ii) an elevated 0800 h plasma level of ACTH (O500 ng/l) in addition to progressive elevations of ACTH (a rise of O30%) on at least three consecutive occasions. Regarding predictive factors for the development of Nelson's syndrome post TBA, current evidence favours the presence of residual pituitary tumour on magnetic resonance imaging (MRI) post transsphenoidal surgery (TSS); an aggressive subtype of corticotrophinoma (based on MRI growth rapidity and histology of TSS samples); lack of prophylactic neoadjuvant pituitary radiotherapy at the time of TBA and a rapid rise of ACTH levels in year 1 post TBA. Finally, more studies are needed to assess the efficacy of therapeutic strategies in Nelson's syndrome, including the alkylating agent, temozolomide, which holds promise as a novel and effective therapeutic agent in the treatment of associated aggressive corticotroph tumours. It is timely to review these controversies and to suggest guidelines for future audit.

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Gamma knife stereotactic radiosurgery of Nelson syndrome

Cited by 40 publications

References 14 publications

Nelson's syndrome: a review of the clinical manifestations, pathophysiology, and treatment strategies

Nelson's syndrome: a review of the clinical manifestations, pathophysiology, and treatment strategies

The Treatment of Cushing's Disease

Nelson's syndrome

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