Relatively high frequencies of loss-of-function SETD2 mutations have been reported in many tumor types, including acute lymphoblastic (10%) and myeloid leukemias (6%), adenocarcinoma of the lung (5%), and above all, clear cell renal cell carcinoma (15–20%), where SETD2 mutations have also been linked to advanced clinical stage at diagnosis and poor prognosis [11]. Here, SETD2 is linked to clear cell renal carcinoma.