PMID- 34333645 OWN - NLM STAT- MEDLINE DCOM- 20211228 LR - 20211228 IS - 1945-7197 (Electronic) IS - 0021-972X (Linking) VI - 106 IP - 12 DP - 2021 Nov 19 TI - Initiating Pancreatic Neuroendocrine Tumor (pNET) Screening in Young MEN1 Patients: Results From the DutchMEN Study Group. PG - 3515-3525 LID - 10.1210/clinem/dgab569 [doi] AB - 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. CI - (c) The Author(s) 2021. Published by Oxford University Press on behalf of the Endocrine Society. FAU - Klein Haneveld, Mirthe J AU - Klein Haneveld MJ AUID- ORCID: 0000-0002-0453-2608 AD - Department of Endocrine Oncology, University Medical Centre Utrecht, 3584 CX, Utrecht, the Netherlands. FAU - van Treijen, Mark J C AU - van Treijen MJC AD - Department of Endocrine Oncology, University Medical Centre Utrecht, 3584 CX, Utrecht, the Netherlands. FAU - Pieterman, Carolina R C AU - Pieterman CRC AD - Department of Endocrine Oncology, University Medical Centre Utrecht, 3584 CX, Utrecht, the Netherlands. AD - Department of Surgical Oncology, Section of Surgical Endocrinology, The University of Texas MD Anderson Cancer Center, Houston, Texas 77030, USA. FAU - Dekkers, Olaf M AU - Dekkers OM AD - Departments of Endocrinology and Metabolism and Clinical Epidemiology, Leiden University Medical Centre, 2333 ZA, Leiden, the Netherlands. FAU - van de Ven, Annenienke AU - van de Ven A AD - Department of Endocrinology, Radboud University Medical Center, 6525 GA, Nijmegen, the Netherlands. FAU - de Herder, Wouter W AU - de Herder WW AUID- ORCID: 0000-0003-1463-5165 AD - Department of Internal Medicine, Erasmus Medical Center, 3015 GD, Rotterdam, the Netherlands. FAU - Zandee, Wouter T AU - Zandee WT AD - Department of Endocrinology, University of Groningen, University Medical Centre Groningen, 9713 GZ, Groningen, the Netherlands. FAU - Drent, Madeleine L AU - Drent ML AD - Department of Internal Medicine, Section of Endocrinology, Amsterdam UMC, location VU University Medical Centre, 1081 HV, Amsterdam, the Netherlands. FAU - Bisschop, Peter H AU - Bisschop PH AD - Department of Endocrinology and Metabolism, Amsterdam UMC, location Academic Medical Centre, 1105 AZ, Amsterdam, the Netherlands. FAU - Havekes, Bas AU - Havekes B AD - Department of Internal Medicine, Division of Endocrinology, Maastricht University Medical Centre, 6229 HX, Maastricht, the Netherlands. FAU - Vriens, Menno R AU - Vriens MR AD - Department of Endocrine Surgery, University Medical Centre Utrecht, 3584 CX, Utrecht, the Netherlands. FAU - Verrijn Stuart, Annemarie A AU - Verrijn Stuart AA AD - Department of Paediatric Endocrinology, Wilhelmina Children's Hospital, University Medical Centre Utrecht, 3584 EA, Utrecht, the Netherlands. FAU - Valk, Gerlof D AU - Valk GD AUID- ORCID: 0000-0001-5841-8344 AD - Department of Endocrine Oncology, University Medical Centre Utrecht, 3584 CX, Utrecht, the Netherlands. FAU - van Leeuwaarde, Rachel S AU - van Leeuwaarde RS AD - Department of Endocrine Oncology, University Medical Centre Utrecht, 3584 CX, Utrecht, the Netherlands. LA - eng PT - Journal Article PL - United States TA - J Clin Endocrinol Metab JT - The Journal of clinical endocrinology and metabolism JID - 0375362 RN - Non functioning pancreatic endocrine tumor SB - IM MH - Adolescent MH - Adult MH - Age of Onset MH - Aged MH - Child MH - Child, Preschool MH - Databases, Factual MH - Diagnostic Imaging MH - Early Detection of Cancer/*methods MH - Female MH - Follow-Up Studies MH - Humans MH - Male MH - Middle Aged MH - Multiple Endocrine Neoplasia Type 1/*physiopathology MH - Netherlands/epidemiology MH - Neuroendocrine Tumors/*diagnosis/epidemiology MH - Pancreatic Neoplasms/*diagnosis/epidemiology MH - Prognosis MH - Retrospective Studies MH - Survival Rate MH - Tumor Burden MH - Young Adult OTO - NOTNLM OT - age-related penetrance OT - multiple endocrine neoplasia type 1 OT - pancreatic NET OT - surveillance EDAT- 2021/08/02 06:00 MHDA- 2021/12/29 06:00 CRDT- 2021/08/01 21:11 PHST- 2021/04/21 00:00 [received] PHST- 2021/08/02 06:00 [pubmed] PHST- 2021/12/29 06:00 [medline] PHST- 2021/08/01 21:11 [entrez] AID - 6333552 [pii] AID - 10.1210/clinem/dgab569 [doi] PST - ppublish SO - J Clin Endocrinol Metab. 2021 Nov 19;106(12):3515-3525. doi: 10.1210/clinem/dgab569.