Published online Jul 28, 2020. doi: 10.3748/wjg.v26.i28.4036
Peer-review started: April 31, 2020
First decision: July 8, 2020
Revised: June 10, 2020
Accepted: July 16, 2020
Article in press: July 16, 2020
Published online: July 28, 2020
Core tip: Pancreatic Neuroendocrine Tumors (PNETs) can cause severe morbidity due to excessive hormones production and overall tumor mass. The majority of approved therapeutic options in PNETs lack objective response suggesting that there is still a void in the understanding of the biology of this neoplasia. With the rising incidence and the underestimated prevalence of PNETs in the United States, it is paramount to discuss the challenges associated with the study and the management of this intractable disease for better patient outcomes. In this paper we elaborate on the comprehensive challenges and discuss novel and emerging therapeutic target in PNETs.