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 - © 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.
PY - 2020/11/2
Y1 - 2020/11/2
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 survival of patients with MEN1-related lung NETs at long-term follow-up.METHODS: The population based Dutch MEN1 Study Group (DMSG) 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 suspect of lung NETs were identified and followed for a median of 6.6 years. Tumor diameter increased 6.0% per year. The overall 15-yr survival was 78.0% (95% confidence interval (CI): 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 six 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 survival of patients with MEN1-related lung NETs at long-term follow-up.METHODS: The population based Dutch MEN1 Study Group (DMSG) 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 suspect of lung NETs were identified and followed for a median of 6.6 years. Tumor diameter increased 6.0% per year. The overall 15-yr survival was 78.0% (95% confidence interval (CI): 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 six 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.
U2 - 10.1210/clinem/dgaa800
DO - 10.1210/clinem/dgaa800
M3 - Article
C2 - 33135721
JO - Journal of Clinical Endocrinology and Metabolism
JF - Journal of Clinical Endocrinology and Metabolism
SN - 0021-972X
ER -