For example, when compared to similar sporadic tumors, VHL-associated endolymphatic sac tumors are less likely to invade surrounding structures (14), VHL-associated spinal hemangioblastomas are less likely to be clinically symptomatic (15), and resected VHL-associated pancreatic neuroendocrine tumors have a significantly lower rate of recurrence than similar sporadic tumors (16). This evidence concerns the gene VHL and endolymphatic sac tumor.