Surgery for Cushing’s Syndrome: An Historical Review and Recent Ten‐year Experience

Porterfield, John R.; Thompson, Geoffrey B.; Young, William F.; Chow, John T.; Fryrear, Raymond; Heerden, Jon A. van; Farley, David R.; Atkinson, John L.D.; Meyer, Fredric B.; Abboud, Charles F.; Nippoldt, Todd B.; Natt, Neena; Erickson, Dana; Vella, Adrian; Carpenter, Paul C.; Richards, Melanie L.; Carney, J. Aidan; Larson, Dirk; Schleck, Cathy D.; Churchward, Marilyn; Grant, Clive S.

doi:10.1007/s00268-007-9387-6

Cited by 70 publications

(49 citation statements)

References 136 publications

(236 reference statements)

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“…In a series of 18 patients with EC who underwent bilateral adrenalectomy, O'Riordain et al (26) found 5-year survival probability of 39%, with 73% of deaths related directly to metastatic malignant disease. Porterfield et al (25), in a group of 35 subjects who also had bilateral adrenalectomy, reported 5-year survival probability of 51.3%. In a series of 90 patients with EC followed up for a median period of 26 months, Ilias et al (27) demonstrated that subjects with an unknown/occult source survived longer compared with those with an identified tumour and that amongst those with identified tumour, patients with pulmonary EC (excluding small cell lung cancer) survived longest.…”

Section: Discussionmentioning

confidence: 97%

Mortality in Cushing's syndrome: systematic analysis of a large series with prolonged follow-up

Ntali

Asimakopoulou

Siamatras

et al. 2013

European Journal of Endocrinology

135

103

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Objective: In this study, we aim to assess the long-term survival and causes of death in a retrospective cohort study on patients with all aetiologies of endogenous Cushing's syndrome (CS) (except adrenal cancer), presenting to two large tertiary endocrine referral centres, and to identify variables predicting mortality. Results: In total, 418 subjects were identified (311 with Cushing's disease (CD), 74 with adrenal Cushing's (AC) and 33 with ectopic Cushing's (EC)). In CD, the probability of 10-year survival was 95.3% with 71.4% of the deaths attributed to cardiovascular causes or infection/sepsis. SMRs were significantly high overall (SMR 9.3; 95% CI, 6.2-13.4, P!0.001), as well as in all subgroups of patients irrespective of their remission status. In AC, the probability of 10-year survival was 95.5% and the SMR was 5.3 (95% CI, 0.3-26.0) with PZ0.2. Patients with EC had the worst outcome with 77.6% probability of 5-year survival. Conclusions: In this large series of patients with CS and long-term follow-up, we report that in CD the mortality is significantly affected, even after apparently successful treatment. The SMR of patients with AC was high, but this was not statistically significant. The implicated pathophysiological mechanisms for these findings need to be further elucidated aiming to improve the long-term outcome.

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Section: Discussionmentioning

confidence: 97%

Mortality in Cushing's syndrome: systematic analysis of a large series with prolonged follow-up

Ntali

Asimakopoulou

Siamatras

et al. 2013

European Journal of Endocrinology

135

103

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“…The success rate of 67% with first TSS in our series is comparable to that reported in the literature. [23][24][25][26][27][28][29][30][31][32][33][34][35][36][37][38] The main weakness of this study is that in about 8% of the patients the source of ACTH escaped detection. This is possibly due to non-availability of some essential investigations like CRH stimulated IPSS for ACTH determination.…”

Section: Discussionmentioning

confidence: 99%

“…19 The measuring range of cortisol assay was 0.018 μg/dL to 63.4 μg/dL with inter-assay and intra-assay CV <5.6%. Plasma ACTH was determined using two monoclonal antibodies specific for ACTH (ACTH: 9-12 & ACTH: [36][37][38][39]. The measuring range of the ACTH assay was 1-2000 pg/ml with inter-assay and intra-assay CV <6%.…”

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confidence: 99%

Cushing's disease: Results of treatment and factors affecting outcome

Ammini

Bhattacharya

Sahoo

et al. 2011

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ObjectIve: to analyze the therapeutic results of intervention and the factors affecting the outcome of patients with cushing's disease (cD) cared for at the All Indian Institute of Medical science (AIIMs), New Delhi. DesIGN. Patients with cushing's disease treated at a teritiary care centre from january 2000 to December 2009 were prospectively studied. resULts. Ninetyseven patients received treatment for cD during this period. Mean duration of follow-up was 3.4 ± 2.2 (mean±sD) years. eighty-one patients (83.5%) underwent transsphenoidal surgery (tss) as the primary treatment modality. Fifty-four patients (66.7%) went into remission after initial tss; ten (18.5%) of them relapsed after a mean follow-up period of 2.9±2.1 (mean±sD) years. Histopathologic examination of resected tissue showed corticotroph adenoma in 48 of the 54 (88.9%) who went into remission and 17 of the 27 (63.0%) who did not go into remission after the initial tss. sixteen patients with severe hypercortisolism underwent bilateral adrenalectomy (bA) as a life-saving measure which was followed by pituitary surgery 6 to 12 months later. Five patients including one with a large macroadenoma required three or more procedures to achieve eucortisolism. cONcLUsION: Fifty-four out of 81 (66.7%) of our patients with cD had remission following initial tss, ten of whom relapsed later on. sixteen patients unerwent bA as a life-saving procedure. Factors affecting outcome were, age, gender, low dose dexamethasone suppression test cortisol value and histologic confirmation of corticotroph adenoma.

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“…Although generally out of favour (12), laparoscopic TBA can be useful when a corticotrophinoma is undetectable, surgically unresectable or there is recurrence following TSS. In these cases, TBA offers 85-100% success rate in controlling hypercortisolaemia (3,11) and is particularly useful in patients with life-threatening manifestations of hypercortisolism (13).…”

Section: Introductionmentioning

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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Surgery for Cushing’s Syndrome: An Historical Review and Recent Ten‐year Experience

Cited by 70 publications

References 136 publications

Mortality in Cushing's syndrome: systematic analysis of a large series with prolonged follow-up

Mortality in Cushing's syndrome: systematic analysis of a large series with prolonged follow-up

Cushing's disease: Results of treatment and factors affecting outcome

Nelson's syndrome

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