Abstract: Pancreatic neuroendocrine neoplasms (pNENs) is one of the gastrointestinal malignancies of significantly heterogeneous, pathologically classified into well differentiated pancreatic neuro-endocrine tumors (pNETs) and poorly differentiated pancreatic neuroendocrine carcinomas. The prognosis and treatment response of pNETs are primely determined by tumor-intrinsic biolo-gical behavior. Accordingly, surgeons need to operate debulking surgery for low-risk patients, as well as implement adjuvant therapies for those at high-risk of relapse and metastasis after curative resection. Notably, as to two distinct subtypes of patients who suffer primary tumor with diameter less than 2 cm and functional pNETs, oncological benefits and functionally symptomatic control should be considered when formulating surgical strategies. In recent years, advances in next-generation sequencing and organoid technologies have provided practical tools for revealing the gene mutations and tumor microenvironment of pNETs. The four signalling pathways, including mTOR signalling, histone modification, altered telomere length and DNA damage repair pathways, are related to the occurrence and development of pNETs and can be used for the personalization precision therapy of pNETs and guiding the development of new drugs. Empirical therapy and clinical trial studies of pNETs are a continuum of contradictions. Therefore, clinicians need to summarize the rules in treatment and develop disciplines in the summary. Based on relevant literatures, the authors explore the hot issues related to pNENs in recent years, in order to provide reference for the diagnosis and treatment of this disease.