TY - JOUR
T1 - The management of neuroendocrine tumors of the lung in MEN1
T2 - results from the Dutch MEN1 Study Group
AU - Van Den Broek, Medard F M
AU - De Laat, Joanne M
AU - Van Leeuwaarde, Rachel S
AU - Van De Ven, Annenienke C
AU - De Herder, Wouter W
AU - Dekkers, Olaf M
AU - Drent, Madeleine L
AU - Kerstens, Michiel N
AU - Bisschop, Peter H
AU - Havekes, Bas
AU - Hackeng, Wenzel M
AU - Brosens, Lodewijk A A
AU - Vriens, Menno R
AU - Buikhuisen, Wieneke A
AU - Valk, Gerlof D
N1 - Publisher Copyright:
© 2020 The Author(s) 2020. Published by Oxford University Press on behalf of the Endocrine Society. All rights reserved. For permissions, please e-mail: [email protected].
PY - 2021/2/1
Y1 - 2021/2/1
N2 - 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.
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.
KW - lung NET
KW - multiple endocrine neoplasia type 1
KW - surveillance
KW - survival
KW - tumor growth
UR - http://www.scopus.com/inward/record.url?scp=85100358779&partnerID=8YFLogxK
U2 - 10.1210/clinem/dgaa800
DO - 10.1210/clinem/dgaa800
M3 - Article
C2 - 33135721
SN - 0021-972X
VL - 106
SP - e1014-e1027
JO - The Journal of clinical endocrinology and metabolism
JF - The Journal of clinical endocrinology and metabolism
IS - 2
ER -