Evaluation of plasma ACTH in the metyrapone test is insufficient for the diagnosis of secondary adrenal insufficiency.

Objective: To determine whether a single measurement of ACTH instead of less available in daily practice 11-deoxycortisol assay is sufficient to rule out or confirm secondary adrenal insufficiency (SAI) in the short Metyrapone test. Design: A retrospective analysis of diagnostic tests (Metyrapone and Synacthen tests) performed at our Center between 2016 and 2018 in patients with suspicion of secondary adrenal insufficiency. Material and methods: In 103 patients short metyrapone test was performed with assessment of 11-deoxycortisol and ACTH concentration after Metyrapone administered at midnight. In 89 of them short Synacthen (SST) test was also done (1 or/and 250 mcg 1-24ACTH). ROC curves have been performed to evaluate the diagnostic performance of ACTH level in metyrapone test as the predictor of secondary adrenal insufficiency (SAI) analysing sensitivity and specificity for various possible thresholds proposed in literature. Results: 40 (39%) of examined subjects were diagnosed as SAI, basing on post-Metyrapone 11-deoxycortisol concentration below 70 µg/l. In this group ACTH concentration was 128.1 ng/l (95% CI 96.8-159.4) versus 289.9 ng/l (95% CI 249.1-330.9) in patients with proper adrenal response. There was only a moderate positive correlation between ACTH and 11-deoxycortisol concentrations (r=0.5; p<0.05). The best cut off value of ACTH in relation to 11-deoxycortisol serum concentrations was 147 ng/l - with sensitivity of 73.2% and specificity 83.9%. However, plasma ACTH was>200ng/ml (the highest threshold proposed in literature) in 8 cases (20%) with positive diagnosis of SAI made on the basis of low 11-deoxycortisole and confirmed in short Synacthen test. Conclusion: Our results indicate that for a valuable evaluation of the results of the metyrapone test, the more readily available plasma ACTH assay cannot replace the measurement of 11-deoxycortisol concentrations.

Poor Performance of Angiotensin II Enzyme-Linked Immuno-Sorbent Assays in Mostly Hypertensive Cohort Routinely Screened for Primary Aldosteronism.

Primary aldosteronism (PA) is the most common, but broadly underdiagnosed, form of hormonal hypertension. To improve screening procedures, current biochemical approaches aim to determine newly appreciated angiotensin II (Ang II) and calculate the aldosterone-to-angiotensin II ratio (AA2R). Thus, the aim of this study was to assess the diagnostic performance of these screening tests in comparison to the aldosterone-to-direct renin ratio (ADRR), which is routinely used. Cheap and available ELISA was used for Ang II measurement. To our knowledge, this is the first study of this laboratory method's usage in PA. The study cohort included 20 PA patients and 80 controls. Ang II concentrations were comparable between PA and non-PA patients (773.5 vs. 873.2 pg/mL, p = 0.23, respectively). The AA2R was statistically significantly higher in PA group when compared with non-PA (0.024 vs. 0.012 ng/dL/pg/mL, p < 0.001). However, the diagnostic performance of the AA2R was significantly worse than that of the ADRR (AUROC 0.754 vs. 0.939, p < 0.01). The sensitivity and specificity of the AA2R were 70% and 76.2%, respectively. Thus, the AA2R was not effective as a screening tool for PA. Our data provide important arguments in the discussion on the unsatisfactory accuracy of renin−angiotensin system evaluation by recently repeatedly used ELISA tests.

Adrenal "nonadenoma" - clinical characteristics and risk of malignancy.

INTRODUCTION: There is an increasing number of adrenal tumours discovered incidentally during imaging examinations performed for many different indications. Based on imaging results, it is possible to differentiate benign from malignant adrenal masses, although there is still a number of equivocal imaging findings. Our study presents 77 cases of adrenal tumours in which imaging was inconclusive and the final diagnosis was stated only after surgery and histopathological examination. MATERIAL AND METHODS: Retrospective data analysis: 77 cases of indeterminate adrenal tumours with a presumptive diagnosis of "nonadenoma" in patients operated within a 16-year period (2004-2019). None of the patients had a history of malignancy, and all tumours were hormonally inactive. On contrast-enhanced computed tomography (CT), the native density of all tumours was higher than 10 Hounsfield Units (HU), and the absolute percentage washout (APW) and relative percentage washout (RPW) were lower than 60% and 40%, respectively. RESULTS: The most common findings were adrenal adenoma (25.9%), macronodular adrenal hyperplasia (16.9%), ganglioneuroma (15.6%), and haemorrhage with posthaemorrhagic changes (13%). In total, there were 12 various histopathological diagnoses in this group. There were only 2 (2.6%) malignant (adrenal cancer and leiomyosarcoma) and 3 (3.9%) potentially malignant (pheochromocytoma) lesions in this group. CONCLUSIONS: It is often impossible to make a correct diagnosis in a clinical setting until it is histologically verified. "Nonadenoma" adrenal tumours constitute a heterogeneous group including very rare pathologies. The risk of malignancy in indeterminate adrenal tumours is relatively low.

Pitfalls in the diagnostics of aldosterone-producing adrenocortical carcinoma.

Not required for Clinical Vignette.

Corticotropinoma as the underlying cause of intermittent Cushing's syndrome in a patient previously diagnosed with primary pigmented nodular adrenocortical disease.

Not required for Clinical Vignette.

Low-dose etomidate for the management of severe hypercortisolaemia in different clinical scenarios: a case series and review of the literature.

BACKGROUND: Severe Cushing's syndrome (SCS) is associated with acute cardiovascular, metabolic and infectious complications. It is considered an emergency, requiring an immediate diagnosis, together with a broad spectrum of supportive and hypocortisolaemic treatments. Surgical intervention, aimed at removing the source of cortisol or adrenocorticotropic hormone (ACTH), is the optimal treatment in most cases of Cushing's syndrome. However, in hypercortisolaemic states, surgical intervention has high rates of perioperative mortality and morbidity. Oral adrenal steroidogenesis inhibitors, even if more effective in combination, are not always efficient enough or well tolerated. Despite their common use, a more potent, parental, immediate, and thus life-saving, therapy is necessary. METHODS: The authors present three different clinical scenarios of etomidate treatment in patients hospitalized in the third reference endocrinological centre in Poland between 2016 and 2017. RESULTS: Patients with Cushing's disease, ectopic Cushing's syndrome and adrenocortical carcinoma presented with severe hypercortisolaemia and exacerbated cortisol-dependent comorbidities. In these three cases, etomidate acted as an accurate, well tolerated and effective cortisol-lowering drug for several days or even months. Patients were monitored in a general ward setting, and no side effects of the therapy were observed. CONCLUSIONS: In doses far lower than those used for anaesthesia, etomidate works as a useful cortisol-lowering therapy in patients intolerant to or unable to take oral medications. Additionally, if urgent, the most potent and effective medical intervention is necessary, and clinicians should be aware of such a therapeutic option.

Prognostic factors in adrenocortical carcinoma: data from a large Polish series.

Introduction Adrenocortical carcinoma (ACC) is a rare malignancy, associated with poor outcome and few therapeutic options. Despite increasing attention, the knowledge about the clinical course and treatment of these tumors is limited. Objectives Survival rates in ACC are still low and the percentage of relapse is high. Thus, it is crucial to identify the prognostic factors of overall survival (OS) and recurrence­free survival (RFS). Patients and methods This was a retrospective analysis of 66 patients diagnosed with ACC between 2002 and 2015. Results The median OS was 43.5 months, 78.19 months for stage I + II, 22.95 months for stage III, and 19.54 months for stage IV ACC. Older age, stage IV ACC, margin status R2, and no mitotane treatment were associated with poor OS. Low Ki67 and mitotic indices were related to improved OS in a univariate analysis. The median RFS was 101.1 months. Disease recurrence after potentially curative surgery was reported in 1 patient (25%) with stage I, 12 patients (46%) with stage II, and 9 patients (45%) with stage III ACC. Male sex and no mitotane treatment were associated with a reduced RFS in a multivariate analysis and higher Ki67 and mitotic indices in the univariate analysis. Conclusions Ki67 and mitotic indices should be considered as prognostic factors when planning the adjuvant treatment of ACC. Mitotane treatment may be independently associated with better outcomes regardless of the tumor stage.

A large tumour of the left atrium - A 10-year follow-up.

Benign myxomas are the most common primary tumors in the left atrium of the heart, and they usually require urgent operative management. However, with the constant aging of the population and treatment of patients with severe concomitant diseases, an interesting question is the efficacy of the conservative approach. We present a case of a 75-year-old woman who was previously diagnosed with a left atrial myxoma, underwent an operation to resect it and then developed a recurrent tumor at the site of resection, which was found by control echocardiography 1.5 years later. She has been observed for over 10 years, as she repeatedly refused reoperation. In the setting of this example of a relatively favorable clinical course of left atrium myxoma, we discuss the current knowledge about the natural history of these tumors and the role of echocardiography in predicting their growth and complications.

Severe thrombocytopenia soon after drug-eluting stent implantation in ST-elevation myocardial infarction.

Thrombocytopenia is among the most frequent haematological issues in patients hospitalised with myocardial infarction. We discuss the case of a 77-year-old male who was admitted to hospital on the 4(th) hour of ST-elevation myocardial infarction of the anterior wall. A percutaneous coronary intervention to critically stenoted initial segment of the anterior descending branch was performed. A few days later he started developing symptomatic thrombocytopenia. On the course of his long hospitalization we faced many important problems, concerning both diagnosis and treatment of these co-existing conditions.