PMID- 34352379 OWN - NLM STAT- MEDLINE DCOM- 20211103 LR - 20211103 IS - 1556-1380 (Electronic) IS - 1556-0864 (Linking) VI - 16 IP - 11 DP - 2021 Nov TI - Well-Differentiated Bronchopulmonary Neuroendocrine Tumors: More Than One Entity. PG - 1810-1820 LID - S1556-0864(21)02335-2 [pii] LID - 10.1016/j.jtho.2021.07.020 [doi] AB - INTRODUCTION: Until now, well-differentiated bronchopulmonary neuroendocrine tumors (bpNET) occurring either sporadically (sp-bpNET) or in the context of multiple endocrine neoplasia type 1 (MEN1) and diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) are regarded as similar entities. However, in contrast to sp-bpNET: MEN1-related and DIPNECH-related bpNET rarely metastasize or lead to bpNET-related death. We aimed to describe and compare the course of the disease of sp-bpNET, DIPNECH- and MEN1-related bpNET. METHODS: All patients with histologically confirmed MEN1-related bpNET from the DutchMEN Study Group database (1990-2017), patients with resected sp-bpNET and DIPNECH patients referred to a Dutch European Neuroendocrine Tumor Society center between 2000 and 2018 were included. Fisher's exact test was used for comparison between groups. The primary end point was disease-specific mortality (DSM). Kaplan-Meier and logrank test were used to compare survival. Cox regression was used to identify risk factors for DSM in the sp-bpNET subgroup. RESULTS: We included 112 sp-bpNET, 29 MEN1, and 27 DIPNECH patients. Tumor classification was similar across subgroups. A total of 20 patients (18%) with sp-bpNET died because of bpNET, compared with none in the MEN1 group and DIPNECH group. Median disease-specific survival was 12.3 (confidence interval: 6.3-18.3) years for patients with sp-bpNET, and not estimable for the other subgroups (p < 0.001). Differences in baseline characteristics did not explain worse survival in sp-bpNET. Tumor classification and age at diagnosis were independent risk factors for DSM in sp-bpNET. CONCLUSIONS: Patients with sp-bpNET have a significantly higher DSM compared with MEN1 or DIPNECH-related bpNET, unexplained by differences in baseline characteristics. This implies that not all bpNET are similar entities. CI - Copyright (c) 2021 International Association for the Study of Lung Cancer. Published by Elsevier Inc. All rights reserved. FAU - van den Broek, Medard F M AU - van den Broek MFM AD - Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, The Netherlands. FAU - Levy, Sonja AU - Levy S AD - Department of Medical Oncology, Netherlands Cancer Institute, Amsterdam, The Netherlands. FAU - Buikhuisen, Wieneke A AU - Buikhuisen WA AD - Department of Thoracic Oncology, Netherlands Cancer Institute, Amsterdam, The Netherlands. FAU - Dijke, Kim AU - Dijke K AD - Department of Medical Oncology, Netherlands Cancer Institute, Amsterdam, The Netherlands. FAU - Hartemink, Koen J AU - Hartemink KJ AD - Department of Surgical Oncology, Netherlands Cancer Institute, Amsterdam, The Netherlands. FAU - van Leeuwaarde, Rachel S AU - van Leeuwaarde RS AD - Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, The Netherlands. FAU - Vriens, Menno R AU - Vriens MR AD - Department of Endocrine Surgical Oncology, University Medical Center Utrecht, Utrecht, The Netherlands. FAU - Tesselaar, Margot E T AU - Tesselaar MET AD - Department of Medical Oncology, Netherlands Cancer Institute, Amsterdam, The Netherlands. FAU - Valk, Gerlof D AU - Valk GD AD - Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, The Netherlands. Electronic address: g.d.valk@umcutrecht.nl. CN - DutchMEN Study Group LA - eng PT - Journal Article DEP - 20210802 PL - United States TA - J Thorac Oncol JT - Journal of thoracic oncology : official publication of the International Association for the Study of Lung Cancer JID - 101274235 SB - IM MH - Humans MH - *Lung Diseases MH - *Lung Neoplasms MH - *Neuroendocrine Tumors MH - *Precancerous Conditions OTO - NOTNLM OT - Bronchopulmonary neuroendocrine tumor (NET) OT - Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) OT - Multiple endocrine neoplasia type 1 (MEN1) OT - Sporadic OT - Survival FIR - de Herder, Wouter IR - de Herder W FIR - Dekkers, Olaf IR - Dekkers O FIR - van de Ven, Annenienke IR - van de Ven A FIR - Havekes, Bas IR - Havekes B FIR - Drent, Madeleine IR - Drent M FIR - Bisschop, Peter IR - Bisschop P FIR - Zandee, Wouter IR - Zandee W FIR - Valk, Gerlof IR - Valk G EDAT- 2021/08/06 06:00 MHDA- 2021/11/04 06:00 CRDT- 2021/08/05 20:17 PHST- 2021/04/09 00:00 [received] PHST- 2021/06/21 00:00 [revised] PHST- 2021/07/02 00:00 [accepted] PHST- 2021/08/06 06:00 [pubmed] PHST- 2021/11/04 06:00 [medline] PHST- 2021/08/05 20:17 [entrez] AID - S1556-0864(21)02335-2 [pii] AID - 10.1016/j.jtho.2021.07.020 [doi] PST - ppublish SO - J Thorac Oncol. 2021 Nov;16(11):1810-1820. doi: 10.1016/j.jtho.2021.07.020. Epub 2021 Aug 2.