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

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.

Overview publication

TitleThe Management of Neuroendocrine Tumors of the Lung in MEN1: Results From the Dutch MEN1 Study Group
DateJanuary 23rd, 2021
Issue nameThe Journal of Clinical Endocrinology & Metabolism
Issue numberv106.2 pe1014-e1027
DOI10.1210/clinem/dgaa800
Authorsvan den Broek MFM, de Laat JM, van Leeuwaarde RS, van de Ven AC, de Herder WW, Dekkers OM, Drent ML, Kerstens MN, Bisschop PH, Havekes B, Hackeng WM, Brosens LAA, Vriens MR, Buikhuisen WA & Valk GD
MTGsMTG4
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