VHL and nonpapillary renal cell carcinoma: Germline mutations in the von Hippel-Lindau (VHL) gene, a tumor suppressor found on chromosome 3p25, are inherited in an autosomal dominant fashion giving way to the development of a spectrum of tumor types including: renal and pancreatic cysts, clear cell renal cell carcinoma (ccRCC), hemangioblastomas (HB), pheochromocytomas (PCC), retinal HBs, and pancreatic neuroendocrine tumors (pNET) [1–3].