SETD2 is a 2-hit tumor suppressor gene, for loss-of-function mutations and deletions were detected in a series of tumor types, most notably in clear cell renal cell cancer (ccRCC) [11] and high-grade gliomas [12] and subsequently presents in a subset of patients with acute lymphoblastic leukemia [13] and acute myeloid leukemia [14]. The gene discussed is SETD2; the disease is nonpapillary renal cell carcinoma.