2023 Volume 38 Issue 6 Pages 361-366
In 2015, the Japanese Society for Neuroendocrine Tumors (JNETS) published the first clinical practice guidelines for pancreatic and gastrointestinal neuroendocrine neoplasms (NEN) to standardize diagnosis and treatment. NEN is a rare tumor that develops in all organs and presents with various clinical symptoms. Pathological classification is ultimately important for the diagnosis of NEN, and it is necessary to select drug treatment strategies based on the pathological diagnosis. The second edition of revised guidelines was published in 2019 to reflect new trends, including WHO classification revision, the advent of somatostatin receptor scintigraphy, the addition of target diseases for molecularly targeted drugs and the somatostatin analogue lanreotide, and reexamination of surgical indications for non-functional pancreatic NEN. Peptide receptor radionuclide therapy (PRRT) has also emerged. In this article, we review the current status and future prospects of pancreatic NEN management.