Various tumors, such as HB in the CNS, RA, Pheo, RCC, neuroendocrine tumors in the pancreas, renal, and pancreatic cysts, have been found to develop through the same process as that in the inactivation of the VHL gene and subsequent functional loss of the pVHL-elong in B-C (VBC) complex. Here, VHL is linked to neuroendocrine neoplasm.