Abstract: Pancreatic neuroendocrine Neoplasms（pNENs）are a class of heterogeneous pancreatic tumors that originate from the pancreatic neuroendocrine cells.Most of them are sporadic，and the etiology and specific pathogenesis are still unclear.The incidence rate has gradually increased in recent years.According to whether it is accompanied by systemic endocrine symptoms，it can be divided into functional and nonfunctional.Most pNENs are non-functional，and often take the space-occupying change of abdominal organs as the first symptom.The liver is the most common metastatic site.Accurate pathological grading and staging are critical to determining the appropriate treatment.Surgery is the only cure，and systemic treatments such as radiotherapy，targeted therapy，and immunotherapy play an increasingly important role.For patients with distant metastases or advanced patients，the goal of treatment is to improve symptoms and prolong survival，which often requires multidisciplinary collaboration to determine the optimal treatment.This article describes the gradual recognition of pNENs as a rare tumor and the evolution of comprehensive diagnosis and treatment methods.

Key words: pancreatic neuroendocrine neoplasms, diagnosis and treatment, progression