The pituitary before and after adrenalectomy for Cushing's syndrome

Manolas, Konstantinos J; Farmer, Helen; Wilson, H K; Kennedy, Allan Laurence; Joplin, G. F.; Montgomery, D. A. D.; Kennedy, T. L.; Welbourn, Richard

doi:10.1007/bf01655082

Cited by 39 publications

(29 citation statements)

References 33 publications

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“…In a smaller cohort of 39 patients the incidence of Nelson's syndrome was 0% in irradiated and 50% in unirradiated patients at a median follow-up of 53 months (50). Similar results have been reported by others (46)(47)(48). The only reported complication of prophylactic irradiation is hypopituitarism, which occurs in 20-30% of patients at 5 years.…”

Section: Nelson's Syndromesupporting

confidence: 85%

“…Pituitary RT employed as a prophylactic measure to reduce the incidence of Nelson's syndrome results in pituitary tumour control of 91% at a corrected median follow-up of 7.8 years (table 3) (7,(46)(47)(48)(49)(50). In a retrospective study of 56 patients with ACTH-dependent Cushing's syndrome treated by adrenalectomy, 5 out of 20 patients (25%) who received prophylactic radiotherapy and 18 of 36 patients (50%) without RT developed Nelson's syndrome at a median follow-up of 9.1 years (49).…”

Section: Nelson's Syndromementioning

confidence: 99%

See 1 more Smart Citation

Radiotherapy and radiosurgery for Cushing's disease

Minniti

Brada

2007

Arq Bras Endocrinol Metab

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Patients with residual or recurrent Cushing's disease receive external beam radiotherapy (RT) with the aim of achieving long-term tumour control and normalization of elevated hormone levels. Treatment is given either as conventional radiotherapy using conformal techniques or as stereotactic radiotherapy, which is either used as fractionated treatment (SCRT) or as single fraction radiosurgery (SRS). We describe the technical aspects of treatment and report a systematic review of the published literature on the efficacy and toxicity of conventional RT, SCRT and SRS. There are no studies directly comparing the different radiation techniques and the reported results are inevitably of selected patients by investigators with interest in the treatment tested. Nevertheless the review of the published literature suggests better hormone and tumour control rates after fractionated irradiation compared to single fraction radiosurgery. Hypopituitarism represents the most commonly reported late complication of radiotherapy seen after all treatments. Although the incidence of other late effects is low, the risk of radiation injury to normal neural structures is higher with single fraction compared to fractionated treatment. Stereotactic techniques offer more localized irradiation compared with conventional radiotherapy, however longer follow-up is necessary to confirm the potential reduction of long-term radiation toxicity of fractionated SCRT compared to conventional RT. On the basis of the available literature, fractionated conventional and stereotactic radiotherapy offer effective treatment for Cushing's disease not controlled with surgery alone. The lower efficacy and higher toxicity of single fraction treatment suggest that SRS is not the appropriate therapy for the majority of patients with Cushing's disease. RESUMORadioterapia e Radiocirurgia para a Doença de Cushing. Pacientes com doença de Cushing residual ou recorrente recebem radioterapia externa em feixe (RT) com o objetivo de alcançar um controle tumoral prolongado e a normalização dos níveis hormonais elevados. O tratamento é realizado tanto com RT convencional, usando técnicas conformacionais, ou com RT estereotáxica, que é usada tanto como tratamento fracionado (RTF) ou como radiocirurgia em procedimento único (RCU). Descreveremos os aspectos técnicos do tratamento e mostraremos uma revisão sistemática da literatura sobre a eficácia e toxicidade da RT convencional, da RTF e da RCU. Não existem estudos comparando diretamente as diferentes técnicas de radiação, e os resultados reportados são inevitavelmente os de pacientes selecionados pelos investigadores com interesse no tratamento testado. De qualquer maneira, a revisão dos dados publicados sugere que há melhores taxas de controle hormonal e tumoral após RTF em comparação com RCU. O hipopituitarismo representa a complicação tardia mais comumente relatada da RT, vista após todos os tipos de tratamento. Embora a incidência de outros efeitos tardios seja baixa, o risco de a radiação comprometer estrutura...

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Section: Nelson's Syndromesupporting

confidence: 85%

Section: Nelson's Syndromementioning

confidence: 99%

Radiotherapy and radiosurgery for Cushing's disease

Minniti

Brada

2007

Arq Bras Endocrinol Metab

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“…In a further study, 25% of those patients receiving prophylactic pituitary radiotherapy developed Nelson's syndrome compared with 50% of those who did not (7). However, there is a controversy regarding the efficacy of neoadjuvant radiotherapy post TBA surgery, with some series suggesting that lack of prophylactic pituitary radiotherapy is not a risk factor for the subsequent development of Nelson's syndrome (8,16,33,35). There is no current consensus on the use of neoadjuvant pituitary radiotherapy post TBA in patients with Cushing's disease.…”

Section: Administration Of Neoadjuvant Radiotherapy Post Tba Surgerymentioning

confidence: 93%

“…There is very little evidence to support the idea that the presence of a small adrenal remnant post TBA provides any protection from the subsequent development of Nelson's syndrome (35). Adrenal remnant tissue also leaves the potential for recurrence of hypercortisolaemia and its sequelae if ACTH levels remain high.…”

Section: Residual Adrenal Remnant After Tbamentioning

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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“…The prevalence of Nelson's syndrome ranges from 8 to 29% in the largest series with more than 40 patients [2,30,[34][35][36][37][38][39][40][41][42][43], with a time interval between adrenalectomy and the diagnosis of Nelson's syndrome ranging from 0.5 to 24 years. Patients were included between the middle of the fifties until the end of the nineties, with an inclusion time ranging from 9 to 36 years.…”

Section: Prevalencementioning

confidence: 99%

The Nelson's syndrome... revisited

et al. 2004

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Adrenalectomy is a radical therapeutic approach to control hypercortisolism in some patients with Cushing's disease. However it may be complicated by the Nelson's syndrome, defined by the association of a pituitary macroadenoma and high ACTH secretion after adrenalectomy. This definition has not changed since the end of the fifties. Today the Nelson's syndrome must be revisited with new to criteria using more sensitive diagnostic tools, especially the pituitary magnetic resonance imaging. In this paper we will review the pathophysiological aspects of corticotroph tumor growth, with reference to the impact of adrenalectomy. The main epidemiological data on the Nelson's syndrome will be presented. More importantly, we will propose a new pathophysiological and practical approach to this question which attempts to evaluate the Corticotroph Tumor Progression after adrenalectomy, rather than to diagnose the Nelson's syndrome. We will discuss the consequences for the management of Cushing's disease patients after adrenalectomy, and will also draw some perspectives.

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The pituitary before and after adrenalectomy for Cushing's syndrome

Cited by 39 publications

References 33 publications

Radiotherapy and radiosurgery for Cushing's disease

Radiotherapy and radiosurgery for Cushing's disease

Nelson's syndrome

The Nelson's syndrome... revisited

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