In the kidney angiomyolipoma datasets we identified four potentially deleterious variants in nuclear-encoded mitochondrial-associated genes including: ATPase Copper Transporting Beta (ATP7B; c.2173A>T, p.Arg725Trp, VAF:40%), NADH Ubiquinone Oxidoreductase Subunit A8 (NDUFA8; c.284A>G, p.Gln95Arg, VAF: 85%), Glycine C-Acetyltransferase (GCAT; c.1201G>T, p. Gly401Trp, VAF: 89%) and Phosphodiesterase 2A (PDE2A; c.930C>A, p. Asp310Glu, VAF: 12%) (Giannikou et al., 2016). This evidence concerns the gene NDUFA8 and urogenital neoplasm.