While VHL mutations leading to elevated CAIX expression by stabilizing HIF present hallmarks of ccRCC [31], RCN1 distinguishes itself by being involved in ER stress related and secretory pathways, suggesting a role apart from the hypoxia pathway or mutations in chromatin remodeling like BAP1 or PBRM1 [32]. Here, VHL is linked to nonpapillary renal cell carcinoma.