Open AccessInitiating Pancreatic Neuroendocrine Tumor (pNET) Screening in Young MEN1 Patients: Results From the DutchMEN Study Group
Author(s) -
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,
A. A. Verrijn Stuart,
Gerlof D. Valk,
Rachel S. van Leeuwaarde
Publication year - 2021
Publication title -
the journal of clinical endocrinology and metabolism/journal of clinical endocrinology and metabolism
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.206
H-Index - 353
eISSN - 1945-7197
pISSN - 0021-972X
DOI - 10.1210/clinem/dgab569
Subject(s) - men1 , neuroendocrine tumors , medicine , multiple endocrine neoplasia , cohort , population , oncology , endocrine system , biology , hormone , biochemistry , environmental health , gene
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.