Neurosurgical Treatment of Nelson’s Syndrome

Kelly, P; Samandouras, George; Grossman, Ashley; Afshar, F.; Besser, G. M.; Jenkins, Paul J.

doi:10.1210/jc.2002-020299

Cited by 83 publications

(85 citation statements)

References 32 publications

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“…Despite this, hyperpigmentation still occurs in up to 42% (25) and visual field defects in 10-57% of patients with Nelson's syndrome (26). The most consistent and reliable biochemical feature of Nelson's syndrome is a marked elevation of plasma ACTH, which continues to rise after adrenalectomy.…”

Section: Clinical Biochemical and Radiological Featuresmentioning

confidence: 99%

“…Pituitary surgery should be the first-line treatment option for Nelson's syndrome, particularly if there is compression of the optic apparatus (25,42,73) with success rates ranging from 10 to 70% (10,25,55,74). Although the usual approach is transsphenoidal, a transcranial procedure may be adopted if there is extrasellar extension (33% of cases (10)).…”

Section: Pituitary Surgerymentioning

confidence: 99%

“…Successful surgery (with long-term remission and fewer complications) is more likely with small tumours confined to the sellar region (74). Overall mortality following surgical management of Nelson's syndrome is 5% (25,73), and morbidity rates are high: up to 69% develop panhypopituitarism (26); 5% acquire a cranial nerve palsy; 15% have a cerebrospinal fluid (CSF) leak; and 8% develop meningitis (25,73).…”

Section: Pituitary Surgerymentioning

confidence: 99%

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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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Section: Clinical Biochemical and Radiological Featuresmentioning

confidence: 99%

Section: Pituitary Surgerymentioning

confidence: 99%

Section: Pituitary Surgerymentioning

confidence: 99%

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Nelson's syndrome

Barber

Adams²,

Ansorge³

et al. 2010

European Journal of Endocrinology

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“…Pituitary surgery may be performed for corticotroph tumour progression if the anatomy is favourable for such an approach (22,23). Given that the risk is of further tumour expansion an attempt at complete hypophysectomy has been recommended, and is associated with a good long-term control of local tumour growth and lowering of plasma ACTH levels, but at a cost of increased hypopituitarism, including diabetes insipidus (22).…”

Section: Surgicalmentioning

confidence: 99%

“…Given that the risk is of further tumour expansion an attempt at complete hypophysectomy has been recommended, and is associated with a good long-term control of local tumour growth and lowering of plasma ACTH levels, but at a cost of increased hypopituitarism, including diabetes insipidus (22). Despite pituitary surgery some patients will have further progression alternative or adjuvant therapy is indicated (22,23).…”

Section: Surgicalmentioning

confidence: 99%

Nelson's Syndrome

Munir

Newell‐Price

2007

Arq Bras Endocrinol Metab

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Nelson's syndrome is a potentially severe complication of bilateral adrenalectomy performed in the treatment of Cushing's disease, and its management remains difficult. Of all of the features of Nelson's syndrome, the one that causes most concern is the development of a locally aggressive pituitary tumour, which, unusually for pituitary disease, may occasionally cause death from the tumour itself. This feature is especially pertinent given the increasing use in Cushing's disease of laparoscopic bilateral adrenal surgery as a highly effective treatment modality to control cortisol-excess. Despite numerous studies and reports, there is no formal consensus of what defines Nelson's syndrome. Thus, some will define Nelson's syndrome according to the classical description with an evolving pituitary mass after bilateral adrenalectomy, whereas others will rely on increasing plasma ACTH levels, even in the absence of a clear pituitary mass lesion on MRI. These factors need to be borne in mind when considering the reports of Nelson's syndrome, as there is great heterogeneity, and it is likely that overall the modern 'Nelson's syndrome' represents a different disease entity from that of the last century. In the present paper, clinical and epidemiological features of Nelson's syndrome, as well as its treatment modalities, are reviewed.

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Neoplasms of the Endocrine Glands: Pituitary Neoplasms

Gandhi

Post

2017

Holland‐Frei Cancer Medicine

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Overview Pituitary neoplasms represent a phenotypically and pathologically diverse family of tumors. Treatment and classification are both patient and tumor specific as symptoms may derive from abnormal hormone production, compression of adjacent nervous system structures, and, in rare cases, metastases. Transsphenoidal surgery represents a common and well‐tolerated treatment for most pituitary adenomas; however, advances in radiation and chemotherapy have increased treatment options, enhancing treatment efficacy, and eligibility.

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Neurosurgical Treatment of Nelson’s Syndrome

Cited by 83 publications

References 32 publications

Nelson's syndrome

Nelson's syndrome

Nelson's Syndrome

Neoplasms of the Endocrine Glands: Pituitary Neoplasms

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