A distinct PRCC subtype was identified that was characterized by a CpG island methylator phenotype (CIMP-RCC) and associated with early-onset disease, poor survival, and germline or somatic mutation of the fumarate hydratase (FH) gene, and a subset of ChRCC with genomic rearrangements within the TERT promoter region was identified that correlated with highly elevated TERT expression and manifestation of kataegis, uncovering a distinct mechanism of TERT upregulation in cancer. Here, FH is linked to cancer.