Comparison of Preoperative and Postoperative Acth Concentrations After Bilateral Adrenalectomy in Cushing's Disease

Barnett, Anthony; Livesey, J. H.; Friday, Karen E.; Donald, R. A.; Espiner, Eric A.

doi:10.1111/j.1365-2265.1983.tb03215.x

Cited by 41 publications

(41 citation statements)

References 6 publications

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“…However, the evidence is not conclusive, and a correlation between urinary cortisol levels and the risk of subsequent development of Nelson's syndrome has not been demonstrated in some studies (13,30,32). Pre-operative urinary cortisol levels cannot therefore be advocated as a reliable predictive factor for the subsequent development of Nelson's syndrome.…”

Section: High Urinary Cortisolmentioning

confidence: 80%

“…Suboptimal or absent steroid replacement therapy post TBA may increase the risk of developing Nelson's syndrome (41,42) or even result in transformation of a pre-existing adenoma into a more aggressive tumour subtype (3,26). However, most studies have not shown this (3,13,32).…”

Section: Insufficient Exogenous Steroid Replacement Therapy Post Tba mentioning

confidence: 88%

“…A rapid rise in plasma ACTH levels in the year following TBA may be associated with a greater risk of developing Nelson's syndrome (8,30,32), and is probably the best validated predictive factor currently available (probably due to its association with corticotrophinoma progression (15)). However, up to 42% of post-TBA patients have persistently elevated (O200 ng/l) plasma ACTH levels in long-term follow-up studies (8), but only a proportion of these go on to develop Nelson's syndrome calling into question the utility of post-TBA ACTH levels as a predictive factor.…”

Section: Acth Levels In the First Post-operative Yearmentioning

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: High Urinary Cortisolmentioning

confidence: 80%

Section: Insufficient Exogenous Steroid Replacement Therapy Post Tba mentioning

confidence: 88%

Section: Acth Levels In the First Post-operative Yearmentioning

confidence: 99%

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Nelson's syndrome

Barber

Adams²,

Ansorge³

et al. 2010

European Journal of Endocrinology

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“…21 One of the best predictive factors for Nelson's syndrome is high levels of ACTH after adrenalectomy, but no definitive threshold value has been established. 5,18,33,34,51,55,66 A rapid rise of plasma ACTH levels in the first year after bilateral adrenalectomy seems to be a strong predictor because of its association with tumor progression.…”

Section: Predictive Factorsmentioning

confidence: 99%

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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“…No nosso serviço, devido ao pequeno número de casos submetidos a esse procedimento, não foi possível concluirmos quanto à existência de seu efeito protetor. Os dados existentes na literatura também não são conclusivos, alguns estudos indicando efeito benéfico (19)(20)(21) …”

Section: Síndrome De Nelsonunclassified

Síndrome de Nelson: relato de caso

Cukier

Duch

Teixeira

et al. 2007

Arq Bras Endocrinol Metab

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RESUMOO objetivo deste artigo é apresentar e discutir alguns aspectos da patogênese, do diagnóstico clínico, hormonal e radiológico e do tratamento da síndrome de Nelson, com Apresentação do caso: Dra. Priscilla Cukier (residente de 1º ano do Serviço de Endocrinologia e Metabologia -HCFMUSP)Paciente de 27 anos, do sexo masculino, pardo, com ganho de peso, aparecimento de estrias e acne, arredondamento do rosto, hipertensão arterial e cefaléia occipital matutina há 2 meses. Exame físico: fácies cushingóide, acantose negra cervical, obesidade centrípeta, estrias violáceas em abdome, braços e tronco, acne no dorso, PA= 150 x 100 mmHg e IMC= 33,6kg/m 2 . Avaliação laboratorial: cortisol sérico basal= 38 µg/dl, cortisol sérico 24 horas= 23,1 µg/dl, cortisol sérico após 1 mg dexametasona overnight= 35 µg/dl, cortisol sérico após 8 mg dexametasona overnight= 12 µg/dl, cortisol urinário= 1.217 µg/24 horas e ACTH plasmático= 30,6 pg/ml (normal < 60 pg/ml). Avaliação radiológica: Tomografia computadorizada (TC) crânio: sela balonizada, parcialmente vazia, com imagem sugestiva de microadenoma que deprimia o assoalho e invadia, discretamente, o seio esfenoidal; TC

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Comparison of Preoperative and Postoperative Acth Concentrations After Bilateral Adrenalectomy in Cushing's Disease

Cited by 41 publications

References 6 publications

Nelson's syndrome

Nelson's syndrome

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

Síndrome de Nelson: relato de caso

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