Endocrine Abstracts

Adrenocortical carcinoma (ACC) is a rare endocrine tumour with a generally poor prognosis. Mitotane is the only approved drug for treatment of advanced ACC (“palliative” setting). However, response is relatively poor with up to 20% of patients experiencing objective response (Megerle 2018). Moreover, adjuvant treatment with mitotane is frequently recommended for patients at high risk of disease recurrence after surgery (Terzolo 2007, Fassnacht 2018). Of note, mitotan...

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...

Background: Adrenocortical carcinoma (ACC) has an aggressive but variable behaviour. ENSAT tumour stage and Ki67 proliferation index are used to predict clinical outcome but they are limited in distinguishing patients with best outcome. We aimed to investigate the prognostic role of clinical/histopathological parameters alone or in combination according to previously proposed points-based score (mGRAS, Lippert JCEM 2018).Methods: We assessed 112 patients...

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...

Background: The chemokine receptor CXCR4 and its associate receptor CXCR7, that modulates CXCR4 function, have been associated with tumor progression and metastasis in human cancers. In ACC, Ki67 index and ENSAT stage are the most important prognostic parameters.Objective: To assess expression of CXCR4 and CXCR7 in adrenal cancer and correlate results with clinical outcome.Methods: CR expression was assessed by immunohistochemistry...

Background: Adrenocortical tumors consist of benign adenomas (ACA) and highly malignant carcinomas (ACC). Dysregulation of the Notch signalling pathway is implicated in several cancers with oncogenic or tumor suppressor functions. JAG1 is a Notch1 ligand of the Jagged family and a common target gene for Notch and Wnt/β catenin pathways. It has been reported that upregulated expression of JAG1 enhances cell proliferation in ACC.Material and methods: ...

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...

Background: Adrenocortical tumors consist of benign adenomas (ACA) and highly malignant carcinomas (ACC) with a still incompletely understood pathogenesis. Our aim was to test, whether there is evidence for an adenoma-carcinoma sequence.Patients and methods: High-resolution single nucleotide polymorphism (SNP) microarrays (Affymetrix SNP 6.0) were used to detect copy number alterations (CNAs) and copy neutral losses of heterozygosity (cnLOH) in 46 adreno...

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...