TY - JOUR
T1 - The Management of Neuroendocrine Tumors of the Lung in MEN1
T2 - Results From the Dutch MEN1 Study Group
AU - van de Broek, M. F.M.
AU - de Laat, J.M.
AU - van Leeuwaarde, R.S.
AU - van de Ven, A.C.
AU - De Herder, Wouter W.
AU - Dekkers, O.M.
AU - Drent, ML
AU - Kerstens, M.N.
AU - Bisschop, P.H.L.T.
AU - Havekes, B.
AU - Hackeng, Wenzel M
AU - Brosens, Lodewijk A.A.
AU - Vriens, M.R.
AU - Buikhuisen, W.A.
AU - Valk, G.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: journals.permissions@oup.com.
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 - https://doi.org/10.1210/clinem/dgaa800
DO - https://doi.org/10.1210/clinem/dgaa800
M3 - Article
C2 - 33135721
SN - 0021-972X
VL - 106
SP - E1014-E1027
JO - Journal of clinical endocrinology and metabolism
JF - Journal of clinical endocrinology and metabolism
IS - 2
ER -