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

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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 and Metabolism
Issue number12
Early online date1 Aug 2021
Publication statusPublished - 1 Dec 2021

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Publisher Copyright:
© 2021 The Author(s) 2021. Published by Oxford University Press on behalf of the Endocrine Society.


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