Clinical features and management of ectopic ACTH syndrome at a single institute in Japan

Doi, Masaru; Sugiyama, Toru; Izumiyama, Hajime; Yoshimoto, Takanobu; Hirata, Yukio

doi:10.1507/endocrj.k10e-265

Cited by 55 publications

(62 citation statements)

References 38 publications

(70 reference statements)

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“…In particular, Ilias et al (13) documented diabetes in half of 90 patients with ectopic CS, while Isidori et al (9) described 37.5% prevalence in a series of 40 patients. However, two other smaller studies on patients with ectopic CS recently described diabetes mellitus in 75-80% of cases (14,15), consistent with our results in a larger cohort. Accordingly, in our study, a diabetologist was consulted more often by patients who were subsequently diagnosed with ectopic CS than those with other aetiologies.…”

Section: Discussionsupporting

confidence: 93%

The European Registry on Cushing's syndrome: 2-year experience. Baseline demographic and clinical characteristics

Valassi

Santos

Yaneva

et al. 2011

European Journal of Endocrinology

333

267

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Objective: The European Registry on Cushing's syndrome (ERCUSYN) is designed to collect prospective and follow-up data at EU level on Cushing's syndrome (CS). Design and methods: Baseline data on 481 CS patients (390 females, 91 males; mean age (GS.D.): 44 G14 years) collected from 36 centres in 23 countries, including new patients from 2008 and retrospective cases since 2000. Patients were divided into four major aetiologic groups: pituitarydependent CS (PIT-CS) (66%), adrenal-dependent CS (ADR-CS) (27%), CS from an ectopic source (ECT-CS) (5%) and CS from other aetiologies (2%). Results: Proportion of men in the ECT-CS group was higher than in the other groups (P!0.05). The ADR-CS group was older than the PIT-CS (P!0.05). Prevalence of hirsutism (92%) and diabetes (74%) in ECT-CS was higher than in the other groups (P!0.05 and P!0.01 respectively). PIT-CS had more skin alterations, menstrual irregularities and hirsutism than ADR-CS (P!0.01). Reduced libido was more prevalent in men than women (P!0.01). Prevalence of spine osteoporosis was higher in men than women (P!0.05), and males had more vertebral and rib fractures than females (52 vs 18% for vertebrae; P!0.001 and 34 vs 23% for ribs; P!0.05). ECT-CS consulted a diabetologist more frequently than ADR-CS (P!0.05), while a gynaecologist was consulted more often by women with PIT-CS or ADR-CS than with ECT-CS (P!0.05). Overall, weight gain was more common in women than men (P!0.01). CushingQoL and EuroQoL visual analogue scale scores did not differ between the groups. Conclusions: The ERCUSYN project demonstrates a heterogeneous clinical presentation of CS at a European level, depending on gender and aetiology.

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Section: Discussionsupporting

confidence: 93%

The European Registry on Cushing's syndrome: 2-year experience. Baseline demographic and clinical characteristics

Valassi

Santos

Yaneva

et al. 2011

European Journal of Endocrinology

333

267

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“…We decided not to follow this approach, because we believe that 100% specificity is difficult to obtain with the CRH test. It has been well documented that a small number of patients with ECS show a clear cortisol and ACTH response to CRH, especially patients with carcinoids (13,14,15,16,17,18,19). Presetting specificity to 100% will always lead to a pronounced reduction in sensitivity.…”

Section: Discussionmentioning

confidence: 99%

ACTH after 15 min distinguishes between Cushing's disease and ectopic Cushing's syndrome: a proposal for a short and simple CRH test

Ritzel

Beuschlein

Berr

et al. 2015

European Journal of Endocrinology

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Objective: The aim of the present study was to validate criteria of corticotropin-releasing hormone (CRH) stimulation and 8 mg dexamethasone suppression (high-dose dexamethasone suppression, HDDS) to distinguish the etiology of ACTH-dependent Cushing's syndrome. Subjects and methods: We retrospectively analyzed cortisol and ACTH after the injection of 100 mg human CRH in confirmed Cushing's disease (CD, nZ78) and confirmed ectopic Cushing's syndrome (ECS, nZ18). Cortisol and ACTH increase (in percentage above basal (% B )) at each time point, maximal increase (Dmax % B ), and area under the curve (AUC % B ) were analyzed using receiver operator characteristics (ROC) curve analyses. Cortisol suppression (% B ) after 8 mg of dexamethasone was evaluated as a supplementary criterion. Results: An increase in ACTH of R43% B at 15 min after CRH was the strongest predictor of CD, with a positive likelihood ratio of 14.0, a sensitivity of 83%, a specificity of 94%, a positive predictive value of 98% and a negative predictive value of 58%. All of the other criteria of stimulated ACTH and cortisol levels were not superior in predicting CD in response to CRH injection. The addition of cortisol suppression by dexamethasone did not increase the discriminatory power. However, the combination of a positive ACTH response at 15 min and a positive HDDS test excluded ECS in all cases. Conclusion: The present findings support the use of plasma ACTH levels 15 min after the injection of human CRH as a response criterion for distinguishing between CD and ECS. The addition of the HDDS test is helpful for excluding ECS when both tests are positive.

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“…EAS patients (36~57%) have positive results on SRS [28][29][30] due to higher binding affinity of octreotide to SSTR-2 and SSTR-5. In fact, 2 cases who showed suppression of ACTH by octreotide in our study were SRS-positive [28]. The present results with more abundant expres-…”

Section: Discussionmentioning

confidence: 99%

Differential gene expression profiles of POMC-related enzymes, transcription factors and receptors between non-pituitary and pituitary ACTH-secreting tumors

et al. 2011

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Clinical features and management of ectopic ACTH syndrome at a single institute in Japan

Cited by 55 publications

References 38 publications

The European Registry on Cushing's syndrome: 2-year experience. Baseline demographic and clinical characteristics

The European Registry on Cushing's syndrome: 2-year experience. Baseline demographic and clinical characteristics

ACTH after 15 min distinguishes between Cushing's disease and ectopic Cushing's syndrome: a proposal for a short and simple CRH test

Differential gene expression profiles of POMC-related enzymes, transcription factors and receptors between non-pituitary and pituitary ACTH-secreting tumors

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