Other metastatic PRCC2 patient with somatic PBRM1 and SETD2 mutations had over 5 years of overall survival with axitinib treatment....The patient without genetic alteration of FH showed a durable response to temsirolimus and axitinib [a tyrosine kinase inhibitor (TKI), which targets vascular endothelial growth factor-receptor 1–3 (VEGFR 1–3)]. We observed PBRM1 and SETD2 mutations in his disease and he had relatively long survival duration.