In the largest retrospective study [27], 32 patients with VHL, including 21 patients harboring pancreatic neoplasm(s) or cyst(s), were treated with various TKIs: sunitinib (n = 12, eight patients with pancreatic involvement), sorafenib (n = 11, eight patients with pancreatic involvement), axitinib (n = 6, four patients with pancreatic involvement) or pazopanib (n = 3, one patient with pancreatic involvement). Here, VHL is linked to pancreatic neoplasm.