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Histologically Proven Bronchial Neuroendocrine Tumors in MEN1: A GTE 51-Case Cohort Study

P. Lecomte * C. Binquet 1, 2 M. Le Bras A. Tabarin C Cardot-Bauters F. Borson-Chazot 3 C Lombard-Bohas 4 Emmanuel Baudin 5 B. Delemer 6 M. Klein B. Vergès 7 T. Aparicio 8 E. Cosson 9, 10 A. Beckers Ph. Caron O. Chabre P Chanson 11 H. Du Boullay I. Guilhem 12 P Niccoli V. Rohmer 13 J. Guigay C. Vulpoi J. Y. Scoazec 14 P Goudet 15 
Abstract : Objective To evaluate the natural history of MEN1-related bronchial endocrine tumors (br-NETs) and to determine their histological characteristics, survival and causes of death. Summary background data br-NETs frequency ranges from 3 to 13% and may reach 32% depending on the number of patients evaluated and on the criteria required for diagnosis. Methods The 1023-patient series of symptomatic MEN1 patients followed up in a median of 48.7 [35.5–59.6] years by the Groupe d’étude des Tumeurs Endocrines was analyzed using time-to-event techniques. Results br-NETs were found in 51 patients (4.8%, [95% CI 3.6–6.2%]) and were discovered by imaging in 86% of cases (CT scan, Octreoscan, Chest X-ray, MRI). Median age at diagnosis was 45 years [28–66]. Histological examination showed 27 (53%) typical carcinoids (TC), 16 (31%) atypical carcinoids (AC), 2 (4%) large cell neuroendocrine carcinomas (LCNEC), 3(6%) small cell neuroendocrine carcinomas (SCLC), 3(6%) TC associated with AC. Overall survival was not different from the rest of the cohort (HR 0.29, [95% CI 0.02–5.14]). AC tended to have a worse prognosis than TC (p = 0.08). Seven deaths were directly related to br-NETs (three AC, three SCLC and one LCNEC). Patients who underwent surgery survived longer (p = 10−4) and were metastasis free, while 8 of 14 non-operated patients were metastatic. There were no operative deaths. Conclusions Around 5% of MEN1 patients develop br-NETs. br-NETs do not decrease overall survival in MEN1 patients, but poorly differentiated and aggressive br-NETs can cause death. br-NETs must be screened carefully. A biopsy is essential to operate on patients in time.
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Contributeur : LNC - université de Bourgogne Connectez-vous pour contacter le contributeur
Soumis le : mardi 29 août 2017 - 15:36:42
Dernière modification le : mardi 1 novembre 2022 - 03:52:13



P. Lecomte, C. Binquet, M. Le Bras, A. Tabarin, C Cardot-Bauters, et al.. Histologically Proven Bronchial Neuroendocrine Tumors in MEN1: A GTE 51-Case Cohort Study. World Journal of Surgery, 2018, 42 (1), pp.143-152. ⟨10.1007/s00268-017-4135-z⟩. ⟨hal-01578666⟩



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