Endocrine Abstracts

Introduction: Adrenocortical carcinoma (ACC) is an aggressive malignancy with a high rate of recurrence. Regular post-operative follow-up imaging is necessary, but associated with high radiation exposure and frequent diagnostic ambiguity. Urine steroid metabolomics has recently been introduced as a novel diagnostic tool for the detection of adrenocortical malignancy in patients with adrenal incidentalomas. Here we present the first clinical study assessing the performance of t...

Objective: Adrenal vein sampling (AVS) is recognised by Endocrine Society guidelines as the only reliable mean to distinguish between aldosterone producing adenomas and bilateral adrenal hyperplasia, the two most common subtypes of primary aldosteronism (PA). However, AVS protocols are not standardised and vary between centres. The objective of the present study was to assess whether the presence or absence of contralateral adrenal (CL) suppression has an impact on the postope...

Objective: The role of surgery for recurrent adrenocortical carcinomas (ACC) is not well defined. Therefore, we evaluated the outcome after surgery for tumor recurrence in patients from the German ACC Registry.Methods: Only patients with first recurrence after initial R0 resection were investigated. Progression–free and overall survival (PFS, OS) after first recurrence were analyzed by Kaplan–Meier method. Cox proportional hazards regression mo...

Adrenocortical carcinoma (ACC) is a rare disease with poor prognosis. Accordingly, in many cases, the attending doctors have no previous experience with the disease. The aim of our study was to evaluate the quality of care in a large number of patients with ACC in Germany. Data from 263 adult patients of the German ACC registry were analyzed with regard to the following parameters: time to diagnosis, hormonal assessment, imaging, histopathological documentation, follow-up. The...

Background: Adrenal masses are discovered in 5% of abdominal imaging scans. Accuracy of currently available imaging tests to diagnose malignancy is poor. In a proof-of-concept study (JCE&M 2011;96(12):3775-84), we had demonstrated 90% sensitivity and specificity in detecting adrenocortical carcinoma (ACC) for urine steroid metabolomics, the combination of mass spectrometry-based steroid profiling and machine learning-based data analysis. This diagnostic performance is supe...

The cytochrome P450 2W1 (CYP2W1) is an orphan enzyme able to activate anticancer pro-drugs and to metabolise endogenous substances as fatty acids and lysophospholipids. Aim of the study was to evaluate the frequency of CYP2W1 polymorphisms in patients with adrenocortical carcinoma (ACC) and correlate it with the sensitivity to mitotane, which represents the only approved drug for the treatment of advanced ACC.Methods: A multicenter retrospective study in...

BackgroundMitotane represents the first-line medical treatment in most patients with adrenocortical carcinoma (ACC). Although adverse effects (AEs) due to mitotane are known to be frequent and may limit treatment, few systematic data are available. Aim of the study was to evaluate the AEs in ACC patients treated with mitotane monotherapy.MethodsA retrospective multicenter study including 311 ACC patients (M:F...

Background: Mitotane is the only drug approved for the treatment of adrenocortical carcinoma (ACC). Although adverse effects (AEs) associated to its use are frequent, detailed information are very limited. Aim was to evaluate the AEs of mitotane monotherapy in ACC patients. Methods: We performed a retrospective multicenter study including 311 ACC patients (F=200, median age 49 yrs) treated with mitotane as first line of medical treatment. Presence and grade of AEs were collect...

Background: Patients with salt-wasting congenital adrenal hyperplasia (CAH) due to classic 21-hydroxylase deficiency require glucocorticoid (GC) and mineralocorticoid (MC) replacement therapy. Recently, it was shown that twice daily modified-release hydrocortisone hard capsules (MRHC, Efmody®, Diurnal Ltd) improved control of CAH with most patients showing good disease control versus standard GC therapy. However, no data has been reported on the renin-angiotens...

Background: Fertility in CAH women is impaired: 0.25 live births vs 1.8 in the UK population and 45% have irregular menses vs 13.6% in healthy women1. Male fertility is also impaired in CAH with oligospermia reported in 48%2. Treatment of infertility usually involves increasing the glucocorticoid dose to normalise adrenal androgens and progesterone to facilitate ovulation and implantation, respectively. Modified-release hydrocortisone (MRHC) capsules, (Ef...