PMID- 30202829 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20200930 IS - 2472-1972 (Electronic) IS - 2472-1972 (Linking) VI - 2 IP - 9 DP - 2018 Sep 1 TI - Diagnosing Nonfunctional Pancreatic NETs in MEN1: The Evidence Base. PG - 1067-1088 LID - 10.1210/js.2018-00087 [doi] AB - In multiple endocrine neoplasia type 1 (MEN1), nonfunctional pancreatic neuroendocrine tumors (NF-pNETs) are the most frequently diagnosed NETs and a leading cause of MEN1-related death. The high prevalence and malignant potential of NF-pNETs outline the need for an evidence-based screening program, as early diagnosis and timely intervention could reduce morbidity and mortality. Controversies exist regarding the value of several diagnostic tests. This systematic review aims to evaluate current literature and amplify an up-to-date evidence-based approach to NF-pNET diagnosis in MEN1. Three databases were systematically searched on the diagnostic value of biomarkers and imaging modalities. Twenty-seven studies were included and critically appraised (modified Quality Assessment of Diagnostic Accuracy Studies). Another 12 studies, providing data on age-related penetrance and tumor growth, were included to assess the optimal frequency and timing of screening. Based on current literature, biomarkers should no longer play a role in the diagnostic process for NF-pNETs, as accuracies are too low. Studies evaluating the diagnostic value of imaging modalities are heterogeneous with varying risks of bias. For the detection of NF-pNETs, endoscopic ultrasound (EUS) has the highest sensitivity. A combined strategy of EUS and MRI seems to be the most useful. Gallium 68 octreotate-DOTA positron emission tomography-CT could be added if NF-pNETs are diagnosed to identify metastasis. Reported growth rates were generally low, and two distinct phenotypes were observed. Surveillance programs should focus on and be adapted to the presence of substantial growth in NF-pNETs. The optimal age to start screening must yet be determined, as insufficient evidence for an evidence-based recommendation was available. FAU - van Treijen, Mark J C AU - van Treijen MJC AD - Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, Netherlands. FAU - van Beek, Dirk-Jan AU - van Beek DJ AD - Department of Endocrine Surgical Oncology, University Medical Center Utrecht, Utrecht, Netherlands. FAU - van Leeuwaarde, Rachel S AU - van Leeuwaarde RS AD - Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, Netherlands. FAU - Vriens, Menno R AU - Vriens MR AD - Department of Endocrine Surgical Oncology, University Medical Center Utrecht, Utrecht, Netherlands. FAU - Valk, Gerlof D AU - Valk GD AD - Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, Netherlands. LA - eng PT - Journal Article PT - Review DEP - 20180731 PL - United States TA - J Endocr Soc JT - Journal of the Endocrine Society JID - 101697997 PMC - PMC6125714 OTO - NOTNLM OT - imaging modalities OT - multiple endocrine neoplasia type 1 OT - pancreatic neuroendocrine tumors OT - screening OT - tumor markers EDAT- 2018/09/12 06:00 MHDA- 2018/09/12 06:01 PMCR- 2018/07/31 CRDT- 2018/09/12 06:00 PHST- 2018/04/03 00:00 [received] PHST- 2018/07/26 00:00 [accepted] PHST- 2018/09/12 06:00 [entrez] PHST- 2018/09/12 06:00 [pubmed] PHST- 2018/09/12 06:01 [medline] PHST- 2018/07/31 00:00 [pmc-release] AID - js_201800087 [pii] AID - 10.1210/js.2018-00087 [doi] PST - epublish SO - J Endocr Soc. 2018 Jul 31;2(9):1067-1088. doi: 10.1210/js.2018-00087. eCollection 2018 Sep 1.