Endocrine Abstracts

Background: Mitotane is regularly used in patients with adrenocortical carcinoma (ACC) adjuvantly, in patients with high risk of recurrence, and in advanced disease. Multiple effects of mitotane result in adrenal insufficiency in virtually all patients. However, it is unclear how frequently the hypothalamic-pituitary-adrenal (HPA) axis is recovering after treatment discontinuation. Here, we aim to investigate the HPA axis after treatment with mitotane.Me...

Background: Serum liquid chromatography–tandem mass spectrometry (LC–MS/MS) steroid profiling is used for the diagnosis of adrenocortical carcinoma (ACC). Guidelines recommend endocrine work-up in addition to radiological imaging for follow-up in ACC, but data on this topic are scarce.Aim: To retrospectively investigate an earlier detection of a recurrent or progressive disease by using endocrine follow-up with LC–MS/MS measurements in com...

Context: Endogenous Cushing syndrome (CS) is associated with hematological abnormalities. Nevertheless, conflicting data have been reported on erythropoiesis. Some studies reported an association between CS and anemia in men, whereas others described erythrocytosis in affected patients. Furthermore, it is unclear whether there are CS subtype-specific changes in red blood cells (RBC) parameters.Objective: To investigate sex and subtype-specific changes in...

Background: The recent ESE-ENSAT guidelines on adrenocortical carcinoma (ACC) indicated new treatment options for patients with “high risk for recurrence” as one of the major research topics for the field. However, since almost no data were available on the use of cytotoxic drugs in an adjuvant setting, no clear recommendation could be given. Here we report our experience with adjuvant platin-based therapy since 2002 at the University of Würzburg and MD Anders...

Adrenocortical carcinoma (ACC) is a rare endocrine malignancy. ACC may rarely occur as part of familial cancer syndromes, but the majority of the cases occur sporadically. A significant proportion of sporadic ACC cases may be preceded by other malignancies and adrenal metastasis from these primary tumours may frequently occur. Herein we present three cases where sporadic ACC was identified in patients with coexistent or previous history of melanoma.Case ...

BackgroundThe ESE-ENSAT guidelines emphasize the role of local therapies and suggest radiotherapy (RT) as an individualized treatment in patients with advanced ACC. However, the evidence for this recommendation is very low. The aim of this study was to retrospectively investigate the efficacy and tolerance of radiation therapy in advanced ACC.MethodsWe screened all patients in five European reference centers ...

Background: Adrenocortical cancer (ACC) is a rare malignant neoplasm with a dismal prognosis, particularly in advanced disease. For these patients only limited therapeutic options are available. Adavosertib (AZD1775) is a potent inhibitor of tyrosine kinase WEE1 that regulates cell cycle checkpoints, slowing cell cycle progression and leading to mitotic entry in the presence of DNA damage. Therefore, its use could potentiate existing DNA damage-based therapies. Here, we evalua...

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: DNA mismatch repair (MMR) maintains genomic integrity and stability. Inactivation of the MMR genes MLH1, PMS2, MSH2, and MSH6 by somatic or germline variants or gene methylation causes MMR deficiency (dMMR) leading to microsatellite instability (MSI) and high tumour mutational burden. In many tumour entities, patients with dMMR/MSI respond well to immune checkpoint inhibitor (ICI) therapy. The clinical relevance of dMMR and MSI i...