ZNRF3 and adrenal cortex carcinoma: It will be important to validate the associations in a second adult cohort, but this could further reflect the known genetic differences in the development of paediatric and adult ACC.36,37 For instance, amplification of chromosome 9q occurs in 90% of paediatric ACC tumours but not in adult tumours.36 Moreover, although upregulation of the Wnt/β-catenin signalling is common in paediatric and adult ACC, mutations in ZNRF3 are only observed in adult tumours.36,37