VHL and primitive neuroectodermal tumor: Moreover, missense VHL pathogenic or likely pathogenic variants weresignificantly associated with adrenal PGL (82.35% vs. 17.65%; p = 0.0001) and PNET(81.81% vs. 18.18%; p = 0.007) compared with non-missense defects (Table 1). In contrast, CNS HBs(90.47% vs. 53.12%; p = 0.004), pancreatic cysts (76.19% vs. 28.12%; p = 0.001), andRCCs (57.14% vs. 12.5; p = 0.001) were significantly more common in patients withnon-missense VHL pathogenic or likely pathogenic variants.