Initiating Pancreatic Neuroendocrine Tumor (pNET) Screening in Young MEN1 Patients: Results From the DutchMEN Study Group

Mirthe J Klein Haneveld, Mark J C van Treijen, Carolina R C Pieterman, Olaf M Dekkers, Annenienke van de Ven, Wouter W de Herder, Wouter T Zandee, Madeleine L Drent, Peter H Bisschop, Bas Havekes, Menno R Vriens, Annemarie A Verrijn Stuart, Gerlof D Valk*, Rachel S van Leeuwaarde

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

Abstract

CONTEXT: Nonfunctioning pancreatic neuroendocrine tumors (NF-pNETs) are highly prevalent and constitute an important cause of mortality in patients with multiple endocrine neoplasia type 1 (MEN1). Still, the optimal age to initiate screening for pNETs is under debate.

OBJECTIVE: The aim of this work is to assess the age of occurrence of clinically relevant NF-pNETs in young MEN1 patients.

METHODS: Pancreatic imaging data of MEN1 patients were retrieved from the DutchMEN Study Group database. Interval-censored survival methods were used to describe age-related penetrance, compare survival curves, and develop a parametric model for estimating the risk of having clinically relevant NF-pNET at various ages. The primary objective was to assess age at occurrence of clinically relevant NF-pNET (size ≥ 20 mm or rapid growth); secondary objectives were the age at occurrence of NF-pNET of any size and pNET-associated metastasized disease.

RESULTS: Five of 350 patients developed clinically relevant NF-pNETs before age 18 years, 2 of whom subsequently developed lymph node metastases. No differences in clinically relevant NF-pNET-free survival were found for sex, time frame, and type of MEN1 diagnosis or genotype. The estimated ages (median, 95% CI) at a 1%, 2.5%, and 5% risk of having developed a clinically relevant tumor are 9.5 (6.5-12.7), 13.5 (10.2-16.9), and 17.8 years (14.3-21.4), respectively.

CONCLUSION: Analyses from this population-based cohort indicate that start of surveillance for NF-pNETs with pancreatic imaging at age 13 to 14 years is justified. The psychological and medical burden of screening at a young age should be considered.

Original languageEnglish
Pages (from-to)3515-3525
Number of pages11
JournalJournal of Clinical Endocrinology & Metabolism
Volume106
Issue number12
DOIs
Publication statusPublished - Dec 2021

Keywords

  • Adolescent
  • Adult
  • Age of Onset
  • Aged
  • Child
  • Child, Preschool
  • Databases, Factual
  • Diagnostic Imaging
  • Early Detection of Cancer/methods
  • Female
  • Follow-Up Studies
  • Humans
  • Male
  • Middle Aged
  • Multiple Endocrine Neoplasia Type 1/physiopathology
  • Netherlands/epidemiology
  • Neuroendocrine Tumors/diagnosis
  • Pancreatic Neoplasms/diagnosis
  • Prognosis
  • Retrospective Studies
  • Survival Rate
  • Tumor Burden
  • Young Adult
  • ENDOCRINE NEOPLASIA TYPE-1
  • INTERVAL-CENSORED-DATA
  • surveillance
  • multiple endocrine neoplasia type 1
  • age-related penetrance
  • pancreatic NET

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