Less Common Types of Pancreatic Neuroendocrine Tumors

Abstract

Less common types of pancreatic neuroendocrine tumors (pNETs) or rare functional pNETs, which include ACTHoma, CRHoma, serotoninoma, calcitoninoma, GHRHoma, GRFoma, parathyroid hormone-related peptide tumor, and ghrelinoma, mainly occur in the pancreas but may also develop in other areas. The clinical features of rare pNETs include specific clinical syndromes associated with ectopic hormone secretion. Because the incidence is extremely low, it is often difficult to define pNETs as a distinct syndrome in most cases. The diagnosis and treatment strategies for less common pNETs are similar to those of other common pNETs.