Initiating pancreatic neuroendocrine tumour (pNET) screening in young MEN1 patients:results from the DutchMEN Study Group

CONTEXT: Non-functioning pancreatic neuroendocrine tumours (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: To assess the age of occurrence of clinically relevant NF-pNETs in young MEN1 patients. PATIENTS AND SETTING: Pancreatic imaging data of MEN1 patients were retrieved from the DutchMEN Study Group database. DESIGN: Interval-censored survival methods were used to describe age-related penetrance, compare survival curves... 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
Reihe/Periodikum: Klein Haneveld , M J , van Treijen , M J C , Pieterman , C R C , Dekkers , O M , van de Ven , A , de Herder , W W , Zandee , W T , Drent , M L , Bisschop , P H , Havekes , B , Vriens , M R , Verrijn Stuart , A A , Valk , G D & van Leeuwaarde , R S 2021 , ' Initiating pancreatic neuroendocrine tumour (pNET) screening in young MEN1 patients : results from the DutchMEN Study Group ' , Journal of Clinical Endocrinology and Metabolism , vol. 106 , no. 12 , pp. 3515-3525 . https://doi.org/10.1210/clinem/dgab569
Sprache: Englisch
Permalink: https://search.fid-benelux.de/Record/base-26671955
Datenquelle: BASE; Originalkatalog
Powered By: BASE
Link(s) : https://hdl.handle.net/11370/d6cd6283-a4c9-4257-81c3-f10312179bbf

CONTEXT: Non-functioning pancreatic neuroendocrine tumours (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: To assess the age of occurrence of clinically relevant NF-pNETs in young MEN1 patients. PATIENTS AND SETTING: Pancreatic imaging data of MEN1 patients were retrieved from the DutchMEN Study Group database. DESIGN: 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. INTERVENTION(S): Not applicable. MAIN OUTCOME MEASURE(S): 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, two of which subsequently developed lymph node metastases. No differences in clinically relevant NF-pNET-free survival were found for sex, timeframe, 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 tumour 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 13-14 is justified. The psychological and medical burden of screening at a young age should be considered.