Status of Surveillance and Nonsurgical Therapy for Small Nonfunctioning Pancreatic Neuroendocrine Tumors

Surg Oncol Clin N Am. 2023 Apr;32(2):343-371. doi: 10.1016/j.soc.2022.10.010.


Pancreatic neuroendocrine tumors (PNETs) occur in < 1/100,000 patients and most are nonfunctioning (NF). Approximately 5% occur as part of multiple endocrine neoplasia type 1. Anatomic and molecular imaging have a pivotal role in the diagnosis, staging and active surveillance. Surgery is generally recommended for nonfunctional pancreatic neuroendocrine tumors (NF-PNETs) >2 cm to prevent metastases. For tumors ≤2 cm, active surveillance is a viable alternative. Tumor size and grade are important factors to guide management. Assessment of death domain-associated protein 6/alpha-thalassemia/mental retardation X-linked and alternative lengthening of telomeres are promising novel prognostic markers. This review summarizes the status of surveillance and nonsurgical management for small NF-PNETs, including factors that can guide management.

Keywords: Active surveillance; EUS-Guided intervention; Multiple endocrine neoplasia type 1; Pancreatic neuroendocrine tumors; Prognostic factors.

Publication types

  • Review

MeSH terms

  • Humans
  • Multiple Endocrine Neoplasia Type 1* / pathology
  • Multiple Endocrine Neoplasia Type 1* / surgery
  • Neuroectodermal Tumors, Primitive*
  • Neuroendocrine Tumors* / pathology
  • Pancreatic Neoplasms* / pathology

Supplementary concepts

  • Non functioning pancreatic endocrine tumor