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. 2021 Jan 23;106(2):e1014-e1027.
doi: 10.1210/clinem/dgaa800.

The Management of Neuroendocrine Tumors of the Lung in MEN1: Results From the Dutch MEN1 Study Group

Medard F M van den Broek  1 , Joanne M de Laat  1   2 , Rachel S van Leeuwaarde  1 , Annenienke C van de Ven  3 , Wouter W de Herder  4 , Olaf M Dekkers  5   6 , Madeleine L Drent  7 , Michiel N Kerstens  8 , Peter H Bisschop  9 , Bas Havekes  10 , Wenzel M Hackeng  11 , Lodewijk A A Brosens  11 , Menno R Vriens  12 , Wieneke A Buikhuisen  13 , Gerlof D Valk  1
Affiliations

The Management of Neuroendocrine Tumors of the Lung in MEN1: Results From the Dutch MEN1 Study Group

Medard F M van den Broek et al. J Clin Endocrinol Metab. .

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 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.

Keywords: lung NET; multiple endocrine neoplasia type 1; surveillance; survival; tumor growth.

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