Abstract

be considered in three groups: the more frequent gastrinomas and insulinomas considered independently and all the rare F-P-NETs (RFTs) considered together and as a separate category (Appendix 1 and table 1 ).Most P-NETs occur as sporadic tumors (non-inherited), although a variable proportion of the different F-P-NETs occurs as part of an inherited syndrome.MEN1 remains the most important inherited condition responsible for 20-30% of gastrinomas and <5% of insulinomas or RFTs [4][5][6][7] ; and uncommon causes of inherited P-NETs include von Hippel Lindau disease (VHL), von Recklinghausen's syndrome (neurofibromatosis 1) and tuberous sclerosis [4,5] .In each of the latter inherited