Clinical research on adrenal cancer
Adrenocortical carcinoma (ACC) and malignant phaeochromocytomas (MPH) are cancer entities associated with unfavourable prognosis and limited treatment options. Due to the rarity of the diseases, diagnosis is often delayed, emphasising the need for better diagnostic tools and expertise to offer optimal care for these patients. So far, the low incidence of these tumours has impeded clinical studies, which have been affected by fragmentation and low cohort sizes. To overcome this, the EU-funded ENS@T-CANCER (European network for the study of adrenal tumours - Structuring clinical research on adrenal cancers in adults) project established a European network that aimed to integrate research on ACC and MPH, working towards the understanding of molecular mechanisms, as well as specific diagnostic and therapeutic tools. Partners collected samples from several patients with rich clinical annotations and biomaterials. Translational studies identified new targets such as SF-1 and the WNT/b-catenin pathway that could result in therapeutic strategies. Significant progress was also made in the standardisation of immunohistochemical techniques and interpretation of morphological tumour characteristics in adrenal cancer diagnosis. For ACC, researchers followed an integrative genomic approach that enabled molecular classification and helped identify molecular markers based on the analysis of methylation alterations in ACC. Regarding MPH, epigenetic markers could aid the differential diagnosis of this rare disease. From a clinical perspective, researchers performed comparative studies between functional imaging and genotype-dependent tumour cell energy metabolism. They also brought together 16 clinical expert centres from seven European countries in intervention clinical trials on MPH to test the efficacy of adjuvant treatment protocols. Taken together, the activities and deliverables of the ENS@T-CANCER consortium should lead to the implementation of new standard treatments. This is expected to improve the clinical outcome of patients with adrenal cancer. Furthermore, the clinical and research network alongside the established registries will support further research in the field.
Keywords
Adrenal cancer, adrenocortical carcinoma, malignant phaeochromocytoma, ENS@T-CANCER, clinical trial
