%0 Journal Article
%T The Management of Neuroendocrine Tumors of the Lung in MEN1: Results From the Dutch MEN1 Study Group.
%A van den Broek MFM
%A de Laat JM
%A van Leeuwaarde RS
%A van de Ven AC
%A de Herder WW
%A Dekkers OM
%A Drent ML
%A Kerstens MN
%A Bisschop PH
%A Havekes B
%A Hackeng WM
%A Brosens LAA
%A Vriens MR
%A Buikhuisen WA
%A Valk GD
%J J Clin Endocrinol Metab
%V 106
%N 2
%D 01 2021 23
%M 33135721
%F 6.134
%R 10.1210/clinem/dgaa800
%X 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.<BR>To assess tumor growth and the survival of patients with MEN1-related lung NETs at long-term follow-up.<BR>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.<BR>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.<BR>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.