Background and aims: Pancreatic neuroendocrine tumors (PNET) arise from uncontrolled proliferation of neuroendocrine cell and further genetic alterations that may induce hormone secretion such as glucagon/insulin/gastrin/VIP. Their incidence is rapidelly growing, especially because of the frequent incidental diagnosis of small asymptomatic non-functionnal neuroendocrine tumors with the widespread use of cross-sectional imaging. The vast majority of pancreatic neuroendocrine tumors are sporadic but up to 5%-10% of them arise from genetic syndromes, the main one being Multiple Endocrine Neopalsm type 1 (MEN1). Appropriate management of patients with PNET is a complex challenge for surgeons, and require extensive medical collaboration. This review aims to summarize major and recent updates regarding the medico-surgical management of PNETs.
Material and methods: Review of pertinent English language literature.
Results: This article provides a concise summary of the clinical presentation, diagnosis, surgical management, alternative treatments and follow up of PNETs.
Conclusion: PNET are a rare, heterogeneous group of neoplasms with a generally favorable prognosis at least compared to pancreatic adenocarcinoma. Surgical resection is the cornerstone of their management, particularly for localized disease, and should always be discussed in multidisciplinary tumor board.
Keywords: Pancreatic neuroendocrine tumor; diagnosis; follow-up; management; pancreatic radiofrequency ablation; pancreatic surgery.