Article (Scientific journals)
Histologically Proven Bronchial Neuroendocrine Tumors in MEN1: A GTE 51-Case Cohort Study.
Lecomte, Pierre; Binquet, C.; Le Bras, M. et al.
2017In World Journal of Surgery
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Abstract :
[en] 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. 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'etude 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.
Disciplines :
Endocrinology, metabolism & nutrition
Author, co-author :
Lecomte, Pierre 
Binquet, C.
Le Bras, M.
Tabarin, A.
Cardot-Bauters, C.
Borson-Chazot, F.
Lombard-Bohas, C.
Baudin, E.
Delemer, B.
Klein, M.
Verges, B.
Aparicio, T.
Cosson, E.
Beckers, Albert ;  Université de Liège - ULiège > Département des sciences cliniques > Endocrinologie
Caron, Ph
Chabre, O.
Chanson, Ph
Du Boullay, H.
Guilhem, I.
Niccoli, P.
Rohmer, V.
Guigay, J.
Vulpoi, C.
Scoazec, J. Y.
Goudet, P.
More authors (15 more) Less
Language :
English
Title :
Histologically Proven Bronchial Neuroendocrine Tumors in MEN1: A GTE 51-Case Cohort Study.
Publication date :
2017
Journal title :
World Journal of Surgery
ISSN :
0364-2313
eISSN :
1432-2323
Publisher :
Springer, Germany
Peer reviewed :
Peer Reviewed verified by ORBi
Available on ORBi :
since 26 September 2017

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