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

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 curve... Mehr ...

Verfasser: Klein Haneveld, Mirthe J
van Treijen, Mark J C
Pieterman, Carolina R C
Dekkers, Olaf M
van de Ven, Annenienke
de Herder, Wouter W
Zandee, Wouter T
Drent, Madeleine L
Bisschop, Peter H
Havekes, Bas
Vriens, Menno R
Verrijn Stuart, Annemarie A
Valk, Gerlof D
van Leeuwaarde, Rachel S
Dokumenttyp: Artikel
Erscheinungsdatum: 2021
Schlagwörter: age-related penetrance / multiple endocrine neoplasia type 1 / pancreatic NET / surveillance / Biochemistry / medical / Endocrinology / Clinical Biochemistry / Diabetes and Metabolism / Journal Article
Sprache: Englisch
Permalink: https://search.fid-benelux.de/Record/base-27457708
Datenquelle: BASE; Originalkatalog
Powered By: BASE
Link(s) : https://dspace.library.uu.nl/handle/1874/443253

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.