PMID- 33135721
OWN - NLM
STAT- MEDLINE
DCOM- 20210920
LR  - 20210920
IS  - 1945-7197 (Electronic)
IS  - 0021-972X (Linking)
VI  - 106
IP  - 2
DP  - 2021 Jan 23
TI  - The Management of Neuroendocrine Tumors of the Lung in MEN1: Results From the 
      Dutch MEN1 Study Group.
PG  - e1014-e1027
LID - 10.1210/clinem/dgaa800 [doi]
AB  - INTRODUCTION: Multiple endocrine neoplasia type 1 (MEN1)-related neuroendocrine 
      tumors (NETs) of the lung are mostly indolent, with a good prognosis. 
      Nevertheless, cases of aggressive lung NET do occur, and therefore the management 
      of individual patients is challenging. AIM: To assess tumor growth and the 
      survival of patients with MEN1-related lung NETs at long-term follow-up. METHODS: 
      The population-based Dutch MEN1 Study Group database (n = 446) was used to 
      identify lung NETs by histopathological and radiological examinations. Tumor 
      diameter was assessed. Linear mixed models and the Kaplan-Meier method were used 
      for analyzing tumor growth and survival. Molecular analyses were performed on a 
      lung NET showing particularly aggressive behavior. RESULTS: In 102 patients 
      (22.9% of the total MEN1 cohort), 164 lesions suspected of lung NETs were 
      identified and followed for a median of 6.6 years. Tumor diameter increased 6.0% 
      per year. The overall 15-year survival rate was 78.0% (95% confidence interval: 
      64.6-94.2%) without lung NET-related death. No prognostic factors for tumor 
      growth or survival could be identified. A somatic c.3127A > G (p.Met1043Val) 
      PIK3CA driver mutation was found in a case of rapid growing lung NET after 6 
      years of indolent disease, presumably explaining the sudden change in course. 
      CONCLUSION: MEN1-related lung NETs are slow growing and have a good prognosis. No 
      accurate risk factors for tumor growth could be identified. Lung NET screening 
      should therefore be based on well-informed, shared decision-making, balancing 
      between the low absolute risk of an aggressive tumor in individuals and the 
      potential harms of frequent thoracic imaging.
CI  - (c) The Author(s) 2020. Published by Oxford University Press on behalf of the 
      Endocrine Society. All rights reserved. For permissions, please e-mail: 
      journals.permissions@oup.com.
FAU - van den Broek, Medard F M
AU  - van den Broek MFM
AD  - Department of Endocrine Oncology, University Medical Center Utrecht, GA Utrecht, 
      The Netherlands.
FAU - de Laat, Joanne M
AU  - de Laat JM
AD  - Department of Endocrine Oncology, University Medical Center Utrecht, GA Utrecht, 
      The Netherlands.
AD  - Department of Internal Medicine, University Medical Center Utrecht, GA Utrecht, 
      The Netherlands.
FAU - van Leeuwaarde, Rachel S
AU  - van Leeuwaarde RS
AD  - Department of Endocrine Oncology, University Medical Center Utrecht, GA Utrecht, 
      The Netherlands.
FAU - van de Ven, Annenienke C
AU  - van de Ven AC
AD  - Department of Endocrinology, Radboud University Medical Center, HB Nijmegen, The 
      Netherlands.
FAU - de Herder, Wouter W
AU  - de Herder WW
AD  - Department of Internal Medicine, Erasmus Medical Center, CA Rotterdam, The 
      Netherlands.
FAU - Dekkers, Olaf M
AU  - Dekkers OM
AD  - Department of Endocrinology and Metabolism, Leiden University Medical Center, RC 
      Leiden, The Netherlands.
AD  - Department of Clinical Epidemiology, Leiden University Medical Center, RC Leiden, 
      The Netherlands.
FAU - Drent, Madeleine L
AU  - Drent ML
AD  - Department of Internal Medicine, Section of Endocrinology, Amsterdam UMC, 
      location VU University Medical Center, MB Amsterdam, The Netherlands.
FAU - Kerstens, Michiel N
AU  - Kerstens MN
AD  - Department of Endocrinology, University Medical Center Groningen, University of 
      Groningen, RB Groningen, The Netherlands.
FAU - Bisschop, Peter H
AU  - Bisschop PH
AD  - Department of Endocrinology and Metabolism, Amsterdam UMC, location Academic 
      Medical Center, DD Amsterdam, The Netherlands.
FAU - Havekes, Bas
AU  - Havekes B
AD  - Department of Internal Medicine, Division of Endocrinology, Maastricht University 
      Medical Center, AZ Maastricht, The Netherlands.
FAU - Hackeng, Wenzel M
AU  - Hackeng WM
AD  - Department of Pathology, University Medical Center Utrecht, GA Utrecht, The 
      Netherlands.
FAU - Brosens, Lodewijk A A
AU  - Brosens LAA
AD  - Department of Pathology, University Medical Center Utrecht, GA Utrecht, The 
      Netherlands.
FAU - Vriens, Menno R
AU  - Vriens MR
AD  - Department of Endocrine Surgical Oncology, University Medical Center Utrecht, GA 
      Utrecht, The Netherlands.
FAU - Buikhuisen, Wieneke A
AU  - Buikhuisen WA
AD  - Department of Thoracic Oncology, The Netherlands Cancer Institute, BE Amsterdam, 
      The Netherlands.
FAU - Valk, Gerlof D
AU  - Valk GD
AD  - Department of Endocrine Oncology, University Medical Center Utrecht, GA 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
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - Child
MH  - Child, Preschool
MH  - Disease Management
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Longitudinal Studies
MH  - Lung Neoplasms/pathology/*surgery
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/pathology/*surgery
MH  - Neuroendocrine Tumors/pathology/*surgery
MH  - Prognosis
MH  - Survival Rate
MH  - Young Adult
OTO - NOTNLM
OT  - lung NET
OT  - multiple endocrine neoplasia type 1
OT  - surveillance
OT  - survival
OT  - tumor growth
EDAT- 2020/11/03 06:00
MHDA- 2021/09/21 06:00
CRDT- 2020/11/02 08:42
PHST- 2020/07/28 00:00 [received]
PHST- 2020/11/03 06:00 [pubmed]
PHST- 2021/09/21 06:00 [medline]
PHST- 2020/11/02 08:42 [entrez]
AID - 5950352 [pii]
AID - 10.1210/clinem/dgaa800 [doi]
PST - ppublish
SO  - J Clin Endocrinol Metab. 2021 Jan 23;106(2):e1014-e1027. doi: 
      10.1210/clinem/dgaa800.