Endocrine Abstracts

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...

Context: Primary aldosteronism (PHA) is the most common cause of secondary hypertension. Distinguishing unilateral (UAH) from bilateral (BAH) and other causes of PHA is one of the greatest challenges in the diagnostic process. Adrenal venous sampling (AVS) is widely considered to be the gold standard for subtype differentiation.Methods: Technical and diagnostic outcome of AVS was analysed retrospectively in 59 patients with PHA who underwent AVS at the U...

Adrenal tumours have an incidence of 2–3% in the general population. Adrenocortical carcinoma (ACC), a highly malignant tumor with a poor prognosis, has an annual incidence of two per million but representation in pre-selected patient cohorts with adrenal masses undergoing surgery is up to 12%. Differentiating adrenocortical adenomas (ACA) from ACC represents a continuous challenge, with unfavourable sensitivities and specificities provided by tumour size, imaging criteri...

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...

Objectives: The TNM classification is a worldwide benchmark for reporting the extent of malignant disease and is intended as a prognostic tool to predict the outcome in patients with cancer. Until 2004, no TNM classification was available for adrenocortical carcinoma (ACC) and different staging systems were used. Due to the rarity of this malignancy, the prognostic value of different staging systems has never been compared directly in a large series of patients.<p class="a...

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...

Adrenocortical carcinoma (ACC) has heterogeneous prognosis and no effective targeted therapies. Pan-genomic studies identified complex molecular patterns related to outcome. Our study aimed at identification of an ‘easy-to-apply’ molecular signature for better personalized prognostic stratification. A total of 107 ACC patients were enrolled. Clinical/histopathological parameters of prognostic relevance were evaluated. Targeted molecular analysis was performed on DNA ...

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...

Recently, somatic, heterozygous mutations in the gene encoding the deubiquitinase USP8 have been identified in 30–60% of corticotroph tumors. These mutations were found to hinder binding of 14-3-3 proteins, increasing its deubiquitinating activity. One substrate is Epidermal Growth Factor Receptor (EGFR), USP8 triggers EGFR recycling and increased EGFR signaling. However, tumors harboring mutations in USP8 are smaller than WT tumors, raising the debate if EGFR, as a poten...

BackgroundAdrenocortical Carcinoma (ACC) is a rare aggressive cancer with a heterogeneous behaviour. Disease surveillance relies on frequent imaging, which has limited sensitivity and results in significant radiation exposure. Aim of the study was to investigate the role of circulating cell-free DNA (ccfDNA) as a biomarker for prognostication and disease monitoring in ACC.MethodsccfDNA was extracted from 1&#8...