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The Management of Neuroendocrine Tumors of the Lung in MEN1: Results From the Dutch MEN1 Study Group
- Source :
- van den Broek , MF , de Laat , JM , Van Leeuwaarde , RS , van de Ven , AC , de Herder , W W , Dekkers , OM , Drent , ML , Kerstens , MN , Bisschop , PH , Havekes , B , Hackeng , WM , Brosens , LAA , Vriens , MR , Buikhuisen , WA & Valk , GD 2021 , ' The Management of Neuroendocrine Tumors of the Lung in MEN1 : Results From the Dutch MEN1 Study Group ' , The Journal of clinical endocrinology and metabolism , vol. 106 , no. 2 , pp. e1014-e1027 .
- Publication Year :
- 2021
-
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.
Details
- Database :
- OAIster
- Journal :
- van den Broek , MF , de Laat , JM , Van Leeuwaarde , RS , van de Ven , AC , de Herder , W W , Dekkers , OM , Drent , ML , Kerstens , MN , Bisschop , PH , Havekes , B , Hackeng , WM , Brosens , LAA , Vriens , MR , Buikhuisen , WA & Valk , GD 2021 , ' The Management of Neuroendocrine Tumors of the Lung in MEN1 : Results From the Dutch MEN1 Study Group ' , The Journal of clinical endocrinology and metabolism , vol. 106 , no. 2 , pp. e1014-e1027 .
- Notes :
- application/pdf, English
- Publication Type :
- Electronic Resource
- Accession number :
- edsoai.on1338021747
- Document Type :
- Electronic Resource