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

Dirk-Jan van Beek, Anna Vera D Verschuur, Lodewijk A A Brosens, Gerlof D Valk, Carolina R C Pieterman*, Menno R Vriens

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

Abstract

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.

Original languageEnglish
Pages (from-to)343-371
Number of pages29
JournalSurgical Oncology Clinics of North America
Volume32
Issue number2
DOIs
Publication statusPublished - Apr 2023

Keywords

  • Humans
  • Multiple Endocrine Neoplasia Type 1/pathology
  • Neuroectodermal Tumors, Primitive
  • Neuroendocrine Tumors/pathology
  • Pancreatic Neoplasms/pathology
  • EUS-Guided intervention
  • Active surveillance
  • Pancreatic neuroendocrine tumors
  • Multiple endocrine neoplasia type 1
  • Prognostic factors

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