VHL and nonpapillary renal cell carcinoma: Affected individuals are at risk of developing various VHL-related manifestations, including retinal hemangioblastoma/angioma (RA), central nervous system hemangioblastoma (CNS-Hbl), clear cell renal cell carcinoma (ccRCC), pancreatic neuroendocrine tumors (pNETs), pheochromocytoma/paraganglioma (PPGL) and endolymphatic sac tumors (ELSTs) (Lonser et al. 2003).