Recently, the International Society of Urological Pathology (ISUP) recognizes clear cell papillary renal cell carcinoma as a distinct epithelial tumor exhibiting characteristics of both pRCC and clear cell renal cell carcinoma (ccRCC) but distinguished from them by genetic differences in the von Hippel-Lindau (VHL) tumor suppressor gene mutation.[4]. The gene discussed is VHL; the disease is neoplasm.