ZNRF3 and adrenal cortex carcinoma: Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy typically poor prognosis.44 ACC is capable of secreting excess adrenocortical hormones, thus compounding morbidity and compromising clinical outcomes.45 An exome sequencing and SNP array analysis in 121 ACCs showed the recurrent homozygous deletions and mutations of RNF203 (ZNRF3), encoding a cell surface E3 ubiquitin ligase, which is located at 22q12.1.46 The RNF203 is the most frequently altered gene (21%) and may be a novel tumor suppressor gene that acts as a negative feedback regulator of WNT/β-catenin signaling.