The management of neuroendocrine tumors of the lung in MEN1: results from the Dutch MEN1 Study Group
Van Den Broek, Medard F M; De Laat, Joanne M; Van Leeuwaarde, Rachel S; Van De Ven, Annenienke C; De Herder, Wouter W; Dekkers, Olaf M; Drent, Madeleine L; Kerstens, Michiel N; Bisschop, Peter H; Havekes, Bas; Hackeng, Wenzel M; Brosens, Lodewijk A A; Vriens, Menno R; Buikhuisen, Wieneke A; Valk, Gerlof D
(2021) The Journal of clinical endocrinology and metabolism, volume 106, issue 2, pp. e1014 - e1027
(Article)
Abstract
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
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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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Keywords: lung NET, multiple endocrine neoplasia type 1, surveillance, survival, tumor growth, Biochemistry, medical, Endocrinology, Biochemistry, Clinical Biochemistry, Endocrinology, Diabetes and Metabolism, Journal Article
ISSN: 0021-972X
Publisher: The Endocrine Society
Note: 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.
(Peer reviewed)