Natural course and survival of neuroendocrine tumors of thymus and lung in MEN1 patients
Journal of Clinical Endocrinology and Metabolism , Volume 99 - Issue 9 p. 3325- 3333
Context: The natural course and survival of neuroendocrine tumors (NETs) of thymus (Th) and lung in multiple endocrine neoplasia type 1 (MEN1) patients are still unknown. CopyrightObjective: Our objective was to assess prevalence, tumor growth, and survival of Th and lung NETs in an unselected MEN1 population with long-term follow-up.Design: This was an observational study. Patients and Methods: A longitudinal study was performed using the Dutch national MEN1 database, including >90% of the Dutch MEN1 population >16 years of age. Patients under care of the Dutch University Medical Centers (1990-2011) (n = 323) were included.Main Outcome Measures: The prevalence and survival of Th and lung NETs were assessed. Linear mixed-models analysis was applied to assess tumor growth with age as a possible confounder and gender, genotype and baseline tumor size as possible effect modifiers.Results: Th NETs occurred in 3.4% of patients, almost exclusively in males with a 10-year survival of25%(95%confidence interval=8%-80%).Athoraciccomputedtomographyscanwasavailable in 188 patients (58.2%). A lung NET was identified in 42 patients (13.0%) with a 10-year survival of 71.1% (95% confidence interval = 51%-100%). Tumor volume of lung NETs increased 17% per year (P < .001) (tumor doubling time 4.5 years). Tumor doubling time in males was 2.5 vs 5.5 years in females (P = .05). Lung NET growth was not associated with genotype or with baseline tumor size (<1 vs ≥1 cm).Conclusion: In MEN1 patients, Th NETs almost exclusively occurred in males and had a very low prevalence and a high mortality. Lung NETs occurred more often than previously thought, had an indolent course, and occurred equally in both sexes. Tumor growth in males was double compared with female patients.
|Journal of Clinical Endocrinology and Metabolism|
|Organisation||Department of Internal Medicine|
De Laat, J.M, Pieterman, C.R.C, Van Den Broek, M.F, Twisk, J.W.R, Hermus, A.R.M.M, Dekkers, O.M, … Valk, G.D. (2014). Natural course and survival of neuroendocrine tumors of thymus and lung in MEN1 patients. Journal of Clinical Endocrinology and Metabolism, 99(9), 3325–3333. doi:10.1210/jc.2014-1560