Abstract:
: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 (>500 ng/l) in addition to progressive elevations of ACTH (a rise of >30%) 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.
journal_name
Eur J Endocrinoljournal_title
European journal of endocrinologyauthors
Barber TM,Adams E,Ansorge O,Byrne JV,Karavitaki N,Wass JAdoi
10.1530/EJE-10-0466subject
Has Abstractpub_date
2010-10-01 00:00:00pages
495-507issue
4eissn
0804-4643issn
1479-683Xpii
EJE-10-0466journal_volume
163pub_type
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journal_title:European journal of endocrinology
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journal_title:European journal of endocrinology
pub_type: 杂志文章,评审
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journal_title:European journal of endocrinology
pub_type: 临床试验,杂志文章,多中心研究
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pub_type: 临床试验,杂志文章
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pub_type: 杂志文章,评审
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journal_title:European journal of endocrinology
pub_type: 临床试验,杂志文章,多中心研究
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更新日期:2004-11-01 00:00:00
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更新日期:2004-06-01 00:00:00
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pub_type: 临床试验,杂志文章
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更新日期:2001-11-01 00:00:00
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journal_title:European journal of endocrinology
pub_type: 杂志文章
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更新日期:1995-04-01 00:00:00
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journal_title:European journal of endocrinology
pub_type: 杂志文章
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更新日期:2010-10-01 00:00:00
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更新日期:2017-11-01 00:00:00
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更新日期:2002-11-01 00:00:00