Endocrine Abstracts

Key learning points• Adrenocortical tumors comprise frequent benign adenomas and rare aggressive carcinomas (ACC).• cAMP/PKA pathway plays a central role in the pathogenesis of cortisol-producing adenomas - associated with Cushing syndrome, while molecular alterations in Wnt/β-catenin, Rb/p53 pathway and chromatin remodeling are frequent in ACCs.<p class="a...

Background: Management of large indeterminate adrenal masses detected during pregnancy is challenging due to the risk of malignancy and the obstetric risks of surgical intervention. The spectrum of endocrine-related complications of Covid-19 is expanding. We present a case that highlights the challenging management of a large adrenal mass during pregnancy and draws attention to a rare complication of Covid-19.Case description: 26 yr-old lady presented wi...

Adrenocortical carcinoma (ACC) is one of the most aggressive endocrine malignancies. By applying a ‘multiple omics’ approach, we recently identified two distinct subgroups of ACC patients, a good prognosis “immune” and bad prognosis “steroid” phenotype.We hypothesized that the steroid phenotype is associated with glucocorticoid-induced suppression that can be ‘rescued’ by reactivating the immune system using immune...

Introduction: Adrenocortical tumors consist in frequent benign adenomas (ACA) and rare highly malignant carcinomas (ACC). BIRC7/livin gene, a member of the inhibitors of apoptosis family, plays an important role in tumorigenesis in a variety of malignancies. Different studies demonstrated that BIRC7 overexpression represent a risk factor for cancer development and progression. The aim of our study was to evaluate the expression of BIRC7 in normal adrenals and adrenocortical tu...

Adrenocortical adenomas are among the most commonly identified human neoplasias, with a prevalence of 2-3% in the population. In some cases they are associated with autonomous cortisol excess that leads to increased morbidity and mortality. Altered cAMP/protein kinase A (PKA) signalling is common in sporadic cortisol-producing adenomas (CPA), mostly caused by somatic mutations in the genes coding for the catalytic subunit α of PKA (PRKACA) or the stimulatory G-pr...

Background: Benign adrenal incidentalomas (AI) are found in 3-5% of adults. All patients should undergo a 1 mg -overnight dexamethasone suppression test (1 mg -DST) to exclude cortisol excess (non-functioning adrenal tumours, NFAT; serum cortisol ≤50 nmol/l) or diagnose possible mild autonomous cortisol secretion (MACS; serum cortisol >50 nmol/l). Current guidelines discourage repeating hormonal work-up in patients with benign AI. However, data underpinning this reco...

Background: Standard treatment for advanced adrenocortical carcinoma (ACC) is mitotane in monotherapy or combined with etoposide, doxorubicin and cisplatin (EDP), yet biomarkers predictive of treatment response are lacking. Inflammation-based scores were proposed as predictors for gemcitabine+capecitabine efficacy, used as second-line in progressive ACC. We investigated the role of inflammation-based scores in predicting response to first-line treatment in advanced ACC.<p ...

Ad Adrenocortical carcinoma (ACC) is an aggressive tumour and treatment remains unsatisfactory in advanced disease. Activation of epithelial to mesenchymal transition (EMT) is considered causative for metastatic spread in a variety of human cancers. Accordingly, new drugs were developed specifically targeting EMT with a focus on hepatocyte growth factor (HGF)/HGF receptor (c-MET) and fibroblast growth factor (FGF)/FGF receptor (FGFR) signalling.We here a...

In a previous study we found mutations in the main catalytic subunit of protein kinase A (PKA Cα) to be responsible for cortisol-secreting adrenocortical adenomas (ACAs). These mutations interfere with the formation of a stable holoenzyme, thus causing constitutive PKA activation. More recently, we identified additional mutations affecting PKA Cα in ACAs associated with overt Cushing syndrome: Ser213Arg_Leu212_Lys214insIle-Ile-Leu-Arg, Cys200_Gly201insVal, Trp197Arg,...

Adrenocortical carcinoma (ACC) is a malignant tumor with poor prognosis and no established therapy in advanced stage. Cisplatin is the most frequently used cytotoxic drug, but even combined with doxorubicin and etoposid, the response rate is <50%. Recently, it has been demonstrated that the excision repair cross complementing group 1 (ERCC1) plays a relevant role in the DNA repairing process, particularly in the correction of platinum-induced DNA adducts. Accordingly, ERCC...