Neuroendocrine tumors (NETs) present a wide spectrum of malignant diseases from rather
benign to very malignant variants. The majority of these tumors are sporadic, but there are
several familial (inherited) syndromes to consider, such as multiple endocrine neoplasia
type 1 and type 2 (MEN-1 and MEN-2), von Hippel-Lindau syndrome (VHL), tuberosclerosis,
and neurofibromatosis syndromes. The MEN-1 gene is mutated not only in MEN-1 families,
but a recent study shows that more than 40% of sporadic pancreatic NETs (PNETs) harbor …

Neuroendocrine cells were discovered in 1870 by Rudolph Heidenhain, who noted that they
could lead to tumors. Siegfried Oberndorfer introduced the term carcinoid in 1907. Since that
time numerous scientists have described tumors that secreted hormones causing a variety of
symptoms. In 1924 Seale Harris described insulinoma, in 1942 William Becker described
glucagonoma, and in 1955 Zollinger and Ellison described gastrinoma. The first reported
case of CCK-oma was described in 2013. The term Neuroendocrine tumors (NET) …