The management of neuroendocrine tumors of the lung in MEN1: results from the Dutch MEN1 Study Group

Medard F M Van Den Broek, Joanne M De Laat, Rachel S Van Leeuwaarde, Annenienke C Van De Ven, Wouter W De Herder, Olaf M Dekkers, Madeleine L Drent, Michiel N Kerstens, Peter H Bisschop, Bas Havekes, Wenzel M Hackeng, Lodewijk A A Brosens, Menno R Vriens, Wieneke A Buikhuisen, Gerlof D Valk

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    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.

    Originele taal-2English
    TijdschriftJournal of Clinical Endocrinology and Metabolism
    StatusE-pub ahead of print - 2-nov-2020

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