Abstract:
:Ectopic Cushing's syndrome usually relates to the ectopic ACTH syndrome (EAS) and represents ∼20% of ACTH-dependent and ∼10% of all types of Cushing's syndrome (CS). Nearly any neuroendocrine or non-endocrine tumours may be associated with EAS, but the more prevalent tumours are bronchial carcinoids, small cell lung carcinomas, pancreatic carcinoids, thymic carcinoids, medullary carcinomas of the thyroid, and phaeochromocytomas. Occult tumours are highly represented in all the series (12-38%) and constitute the more challenging cases of EAS, requiring long term follow-up. The lack of any completely reliable diagnostic test procedure and imaging to clearly reveal the source of EAS suggests that we should adopt a step-by-step multidisciplinary approach for their diagnosis and therapeutic management. Clinical features are often similar in ACTH-dependent CS, but the rapid onset and progress may suggest an ectopic source. A combination of biochemical tests and imaging studies seems the most appropriate approach for the prompt identification of EAS, even if there are several pitfalls to be avoided along the way. The most appropriate management for cure of EAS, when its source is identified, is surgical excision after controlling the hypercortisolaemia by inhibitors of cortisol secretion and other newer modalities alone or in combination; bilateral adrenalectomy remains an alternative option. Tumour histology, the presence of metastases and the effective control of hypercortisolaemia affect mortality and morbidity. If a source repeatedly fails to be found, the prognosis is often favourable but the identification of a malignant tumour should still be sought during life-long follow-up to avoid the calamity of misdiagnosis.
journal_name
Rev Endocr Metab Disordjournal_title
Reviews in endocrine & metabolic disordersauthors
Alexandraki KI,Grossman ABdoi
10.1007/s11154-010-9139-zsubject
Has Abstractpub_date
2010-06-01 00:00:00pages
117-26issue
2eissn
1389-9155issn
1573-2606journal_volume
11pub_type
杂志文章,评审abstract::This paper provides evidence that primary aldosteronism is likely much less common than is believed by the other authors of this collection. The basis for this contrarian view is simple: there is no way to know the true prevalence of PA because of the uncertain validity of all of the diagnostic procedures used to find...
journal_title:Reviews in endocrine & metabolic disorders
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journal_title:Reviews in endocrine & metabolic disorders
pub_type: 杂志文章,评审
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journal_title:Reviews in endocrine & metabolic disorders
pub_type: 杂志文章,评审
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journal_title:Reviews in endocrine & metabolic disorders
pub_type: 杂志文章,评审
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journal_title:Reviews in endocrine & metabolic disorders
pub_type: 杂志文章,评审
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journal_title:Reviews in endocrine & metabolic disorders
pub_type: 杂志文章,评审
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journal_title:Reviews in endocrine & metabolic disorders
pub_type: 杂志文章,评审
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更新日期:2013-03-01 00:00:00
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journal_title:Reviews in endocrine & metabolic disorders
pub_type: 杂志文章,评审
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更新日期:2009-09-01 00:00:00
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journal_title:Reviews in endocrine & metabolic disorders
pub_type: 杂志文章,评审
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更新日期:2010-03-01 00:00:00
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journal_title:Reviews in endocrine & metabolic disorders
pub_type: 杂志文章,评审
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更新日期:2012-06-01 00:00:00
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journal_title:Reviews in endocrine & metabolic disorders
pub_type: 杂志文章,评审
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更新日期:2017-12-01 00:00:00
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pub_type: 杂志文章,评审
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更新日期:2016-12-01 00:00:00
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journal_title:Reviews in endocrine & metabolic disorders
pub_type: 杂志文章,评审
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更新日期:2020-06-03 00:00:00
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pub_type: 杂志文章,评审
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更新日期:2007-12-01 00:00:00
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pub_type: 杂志文章,评审
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pub_type: 杂志文章,评审
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pub_type: 杂志文章,评审
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更新日期:2008-06-01 00:00:00
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journal_title:Reviews in endocrine & metabolic disorders
pub_type: 杂志文章,评审
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更新日期:2008-09-01 00:00:00
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pub_type: 杂志文章,评审
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更新日期:2010-03-01 00:00:00
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journal_title:Reviews in endocrine & metabolic disorders
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更新日期:2013-12-01 00:00:00
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journal_title:Reviews in endocrine & metabolic disorders
pub_type: 杂志文章,评审
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pub_type: 杂志文章,评审
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