World Journal of Surgery

, Volume 42, Issue 1, pp 143–152 | Cite as

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

  • P. Lecomte
  • C. Binquet
  • M. Le Bras
  • A. Tabarin
  • C. Cardot-Bauters
  • F. Borson-Chazot
  • C. Lombard-Bohas
  • E. Baudin
  • B. Delemer
  • M. Klein
  • B. Vergès
  • T. Aparicio
  • E. Cosson
  • A. Beckers
  • Ph. Caron
  • O. Chabre
  • Ph. Chanson
  • H. Du Boullay
  • I. Guilhem
  • P. Niccoli
  • V. Rohmer
  • J. Guigay
  • C. Vulpoi
  • J. Y. Scoazec
  • P. Goudet
Original Scientific Report



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.


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.


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.


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.



The authors thank Annie Costa and Sandrine Daniel for the data management and Philip Bastable for checking the English.

Compliance with ethical standards

Conflict of interest

The authors have nothing to disclose.


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Copyright information

© Société Internationale de Chirurgie 2017

Authors and Affiliations

  • P. Lecomte
    • 1
  • C. Binquet
    • 2
    • 3
    • 4
  • M. Le Bras
    • 5
  • A. Tabarin
    • 6
  • C. Cardot-Bauters
    • 7
  • F. Borson-Chazot
    • 8
    • 9
  • C. Lombard-Bohas
    • 10
  • E. Baudin
    • 11
  • B. Delemer
    • 12
  • M. Klein
    • 13
  • B. Vergès
    • 14
  • T. Aparicio
    • 15
  • E. Cosson
    • 16
  • A. Beckers
    • 17
  • Ph. Caron
    • 18
  • O. Chabre
    • 19
  • Ph. Chanson
    • 20
  • H. Du Boullay
    • 21
  • I. Guilhem
    • 22
  • P. Niccoli
    • 23
  • V. Rohmer
    • 24
  • J. Guigay
    • 25
  • C. Vulpoi
    • 26
  • J. Y. Scoazec
    • 27
  • P. Goudet
    • 28
  1. 1.Service d’Endocrinologie, Nutrition, Diabétologie ENDCentre Hospitalier Regional Universitaire de ToursToursFrance
  2. 2.CIC, module épidémiologie clinique/essais cliniquesCentre Hospitalier Universitaire de DijonDijonFrance
  3. 3.INSERM U866Universite de BourgogneDijonFrance
  4. 4.UMR866, équipe EPICADUniversité de Bourgogne-Franche ComtéDijonFrance
  5. 5.Clinique d’EndocrinologieCentre Hospitalier Universitaire de NantesNantesFrance
  6. 6.Service d’Endocrinologie, Hôpital de Haut LévêqueCentre Hospitalier Universitaire de BordeauxBordeauxFrance
  7. 7.Service de Médecine Interne et EndocrinologieCentre Hospitalier Régional et Universitaire de LilleLilleFrance
  8. 8.Groupement Hospitalier Est, Fédération d’EndocrinologieCentre Hospitalier Universitaire de LyonLyonFrance
  9. 9.Fédération Hospitalo Universitaire d’EndocrinologieUniversité Lyon 1LyonFrance
  10. 10.Service d’Oncologie MédicaleHôpital Edouard HerriotLyonFrance
  11. 11.Département de Médecine Nucléaire et d’Oncologie EndocrinienneGustave RoussyVillejuifFrance
  12. 12.Département d’EndocrinologieCentre Hospitalier Universitaire de ReimsReimsFrance
  13. 13.Service d’EndocrinologieCentre Hospitalier Universitaire de NancyVandœuvre-lès-NancyFrance
  14. 14.Service d’EndocrinologieCentre Hospitalier Universitaire de DijonDijonFrance
  15. 15.Service de Gastro-Entérologie et Cancérologie DigestiveHopital AvicenneBobignyFrance
  16. 16.Polyclinique d’Endocrinologie, Diabétologie, Métabolisme et NutritionHopital Jean VerdierBondyFrance
  17. 17.Service d’EndocrinologieCentre hospitalier universitaire de LiegeLiègeBelgium
  18. 18.Service d’Endocrinologie et Maladies MétaboliquesCentre Hospitalier Universitaire de ToulouseToulouseFrance
  19. 19.Service d’EndocrinologieCentre Hospitalier Universitaire de GrenobleGrenobleFrance
  20. 20.Service d’Endocrinologie et des maladies de la reproductionCentre Hospitalier Universitaire du Kremlin-BicêtreLe Kremlin-BicêtreFrance
  21. 21.Service d’EndocrinologieCentre Hospitalier Metropole SavoieChambéryFrance
  22. 22.Service d’Endocrinologie, Diabétologie, NutritionCentre Hospitalier Universitaire de RennesRennesFrance
  23. 23.Département d’Oncologie MédicaleInstitut Paoli-CalmettesMarseilleFrance
  24. 24.Département d’EndocrinolgieCentre Hospitalier Universitaire d’AngersAngersFrance
  25. 25.Center anti Cancer A. Lacassagne of NiceNiceFrance
  26. 26.Service d’EndocrinologieUniversité de Médecine et Pharmacie “Grigore T.Popa”IaşiRomania
  27. 27.Service de Pathologie Morphologique et MoléculaireGustave RoussyVillejuifFrance
  28. 28.Service de Chirurgie Digestive, Cancérologique et EndocrinienneCentre Hospitalier Universitaire de DijonDijonFrance

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