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

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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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Acknowledgements

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

Author information

Authors and Affiliations

  1. Service d’Endocrinologie, Nutrition, Diabétologie END, Centre Hospitalier Regional Universitaire de Tours, Tours, France

    P. Lecomte

  2. CIC, module épidémiologie clinique/essais cliniques, Centre Hospitalier Universitaire de Dijon, Dijon, France

    C. Binquet

  3. INSERM U866, Universite de Bourgogne, Dijon, France

    C. Binquet

  4. UMR866, équipe EPICAD, Université de Bourgogne-Franche Comté, Dijon, France

    C. Binquet

  5. Clinique d’Endocrinologie, Centre Hospitalier Universitaire de Nantes, Nantes, France

    M. Le Bras

  6. Service d’Endocrinologie, Hôpital de Haut Lévêque, Centre Hospitalier Universitaire de Bordeaux, Bordeaux, France

    A. Tabarin

  7. Service de Médecine Interne et Endocrinologie, Centre Hospitalier Régional et Universitaire de Lille, Lille, France

    C. Cardot-Bauters

  8. Groupement Hospitalier Est, Fédération d’Endocrinologie, Centre Hospitalier Universitaire de Lyon, Lyon, France

    F. Borson-Chazot

  9. Fédération Hospitalo Universitaire d’Endocrinologie, Université Lyon 1, Lyon, France

    F. Borson-Chazot

  10. Service d’Oncologie Médicale, Hôpital Edouard Herriot, Lyon, France

    C. Lombard-Bohas

  11. Département de Médecine Nucléaire et d’Oncologie Endocrinienne, Gustave Roussy, Villejuif, France

    E. Baudin

  12. Département d’Endocrinologie, Centre Hospitalier Universitaire de Reims, Reims, France

    B. Delemer

  13. Service d’Endocrinologie, Centre Hospitalier Universitaire de Nancy, Vandœuvre-lès-Nancy, France

    M. Klein

  14. Service d’Endocrinologie, Centre Hospitalier Universitaire de Dijon, Dijon, France

    B. Vergès

  15. Service de Gastro-Entérologie et Cancérologie Digestive, Hopital Avicenne, Bobigny, France

    T. Aparicio

  16. Polyclinique d’Endocrinologie, Diabétologie, Métabolisme et Nutrition, Hopital Jean Verdier, Bondy, France

    E. Cosson

  17. Service d’Endocrinologie, Centre hospitalier universitaire de Liege, Liège, Belgium

    A. Beckers

  18. Service d’Endocrinologie et Maladies Métaboliques, Centre Hospitalier Universitaire de Toulouse, Toulouse, France

    Ph. Caron

  19. Service d’Endocrinologie, Centre Hospitalier Universitaire de Grenoble, Grenoble, France

    O. Chabre

  20. Service d’Endocrinologie et des maladies de la reproduction, Centre Hospitalier Universitaire du Kremlin-Bicêtre, Le Kremlin-Bicêtre, France

    Ph. Chanson

  21. Service d’Endocrinologie, Centre Hospitalier Metropole Savoie, Chambéry, France

    H. Du Boullay

  22. Service d’Endocrinologie, Diabétologie, Nutrition, Centre Hospitalier Universitaire de Rennes, Rennes, France

    I. Guilhem

  23. Département d’Oncologie Médicale, Institut Paoli-Calmettes, Marseille, France

    P. Niccoli

  24. Département d’Endocrinolgie, Centre Hospitalier Universitaire d’Angers, Angers, France

    V. Rohmer

  25. Center anti Cancer A. Lacassagne of Nice, Nice, France

    J. Guigay

  26. Service d’Endocrinologie, Université de Médecine et Pharmacie “Grigore T.Popa”, Iaşi, Romania

    C. Vulpoi

  27. Service de Pathologie Morphologique et Moléculaire, Gustave Roussy, Villejuif, France

    J. Y. Scoazec

  28. Service de Chirurgie Digestive, Cancérologique et Endocrinienne, Centre Hospitalier Universitaire de Dijon, Dijon, France

    P. Goudet

Authors
  1. P. Lecomte
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  2. C. Binquet
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  3. M. Le Bras
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  19. I. Guilhem
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Correspondence to P. Lecomte.

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Lecomte, P., Binquet, C., Le Bras, M. et al. Histologically Proven Bronchial Neuroendocrine Tumors in MEN1: A GTE 51-Case Cohort Study. World J Surg 42, 143–152 (2018). https://doi.org/10.1007/s00268-017-4135-z

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