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Initiating Pancreatic Neuroendocrine Tumor (pNET) Screening in Young MEN1 Patients:Results from the DutchMEN Study Group
- Source :
- 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 Tumor (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 .
- Publication Year :
- 2021
-
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.
Details
- Database :
- OAIster
- Journal :
- 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 Tumor (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 .
- Notes :
- application/pdf, English
- Publication Type :
- Electronic Resource
- Accession number :
- edsoai.on1313640494
- Document Type :
- Electronic Resource