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

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.

Overview publication

TitleInitiating Pancreatic Neuroendocrine Tumor (pNET) Screening in Young MEN1 Patients: Results From the DutchMEN Study Group
DateAugust 1st, 2021
Issue nameThe Journal of Clinical Endocrinology & Metabolism
Issue numberdoi:10.1210/clinem/dgab569
DOI10.1210/clinem/dgab569
AuthorsKlein Haneveld MJ, van Treijen MJC, Pieterman CRC, Dekkers OM, van de Ven A, de Herder WW, Zandee WT, Drent ML, Bisschop PH, Havekes B, Vriens MR, Verrijn Stuart AA, Valk GD & van Leeuwaarde RS
MTGsMTG4
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