2021-01-23
The Management of Neuroendocrine Tumors of the Lung in MEN1: Results From the Dutch MEN1 Study Group
Publication
Publication
Journal of Clinical Endocrinology and Metabolism , Volume 106 - Issue 2 p. e1014- e1027
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.
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doi.org/10.1210/clinem/dgaa800, hdl.handle.net/1765/134572 | |
Journal of Clinical Endocrinology and Metabolism | |
Organisation | Department of Internal Medicine |
Van Den Broek, M. F., De Laat, J. M., Van Leeuwaarde, R. S., van de Ven, A., de Herder, W., Dekkers, O., … Valk, G. (2021). The Management of Neuroendocrine Tumors of the Lung in MEN1: Results From the Dutch MEN1 Study Group. Journal of Clinical Endocrinology and Metabolism, 106(2), e1014–e1027. doi:10.1210/clinem/dgaa800 |