Endocrine Abstracts

BackgroundAdrenocortical carcinoma (ACC) is a rare disease with often poor prognosis. Previous research has shown that surgery in a Dutch Adrenal Network (DAN) center increases the chance of survival. We aim to explore the determinants and survival of patients with ACC recently treated in the Netherlands both within and outside DAN centers.MethodsWe analyzed retrospectively collected data from 172 adult patie...

Background: One in 5 million children is affected by adrenocortical carcinoma (ACC) each year. Since prognosis for children older than 4 years is limited, clinicians often choose aggressive treatment with etoposide, doxorubicin, cisplatin (EDP) and mitotane after resection. However, little is known about the impact of EDP-mitotane on development in children. We therefore aimed to provide an overview of side-effects and neurocognitive effects of EDP-mitotane in children.<p ...

Introduction: Current medical treatment of adrenocortical carcinoma (ACC) is based on mitotane alone or in combination with cytotoxic chemotherapy. However, very little is known about the pharmacokinetic properties of mitotane and dosing schedules are based on clinical experience only. The aim of this study was to investigate the relationship between mitotane dose and plasma concentration comparing two pre-defined treatment regimens. Secondary objectives were to evaluate safet...

Background: No randomized trials have been conducted in adrenocortical carcinoma (ACC) patients. Treatment recommendations for this rare but aggressive disease have been based on data from small phase II trials. We have now performed the first randomized phase III trial, comparing etoposide, doxorubicin, cisplatin, plus mitotane (EDP-M) against streptozotocin plus mitotane (Sz-M).Methods: Three-hundred and four chemotherapy-naive patients with ACC not am...

Introduction: Adrenocortical carcinoma (ACC) has a reported annual incidence of 0.5–2.0 cases per million individuals. Updated population-based studies on incidence are lacking. The aim of this nationwide survey was to describe the incidence and survival rate of ACC in the Netherlands. Secondary objectives were to evaluate changes in survival rates and in the number of patients undergoing surgery.Methods: All ACC patients registered in the Netherlan...

Introduction: O,p’DDD is the drug of choice for patients with adrenocortical carcinoma (ACC). Monitoring o,p’DDD serum level has been proposed as predictor of tumour response. Measurement of o,p’DDD metabolites involved in the active pathway may provide an improved prediction of o,p’DDD activity. The objective of our study was to (1)to confirm the currently used threshold o,p’DDD serum level of 14mg/l for achieving a tumour response and (2)compare the ...

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: Adjuvant mitotane therapy is frequently used in Europe following surgery for adrenocortical carcinoma (ACC). Management of adjuvant mitotane is mainly empirical and a major open question is the optimal duration of therapy, because no study has ever addressed this issue.Objective: We aimed to assess the outcome of ACC patients who were treated with adjuvant mitotane for at least one year following surgery and then discontinued therapy for othe...

Background: Adrenocortical carcinoma (ACC) has an aggressive but heterogenous behaviour. ENSAT stage and Ki67 proliferation index are used to predict clinical outcome but are limited in distinguishing patients with best outcome. We aimed to validate the prognostic role of clinical and histopathological parameters alone or combined by applying a previously proposed points-based score (mGRAS, Lippert JCEM 2018) to a large multicentre cohort of ACC patients.<p class="abstext"...

Introduction: The prognosis of stages III–IV ACC patients is dismal. The 5-year survival of stage IV ACC ranges between 0 and 13% and no prognostic study has focused on stage III, yet. Several reports suggest a greater heterogeneity of advanced ACC prognosis that initially thought.Aim: The primary objective of our study was to analyse the prognostic factors of overall survival of the subgroup of advanced unresectable stage III–IV ACC patients c...