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)

1 Equipe EPICAD (LNC - U1231)
2 CIC-EC - Centre d'Investigation Clinique 1432 (Dijon) - Epidemiologie Clinique/Essais Cliniques
3 UNICANCER/CRCL - Centre de Recherche en Cancérologie de Lyon
4 Service d'Oncologie Médicale [Centre hospitalier Lyon Sud - HCL]
5 Médecine nucléaire
6 Service d'Endocrinologie - Diabète - Nutrition [Reims]
7 Service d'Endocrinologie, Diabétologie et Maladies Métaboliques (CHU de Dijon)
8 Service de Gastro-entérologie [Avicenne]
9 CRESS - U1153 - Equipe 3: EREN- Equipe de Recherche en Epidémiologie Nutritionnelle
10 Service d'endocrinologie, diabétologie et nutrition
11 Signalisation Hormonale, Physiopathologie Endocrinienne et Métabolique
12 Centre Hospitalier Universitaire de Rennes [CHU Rennes] = Rennes University Hospital [Ponchaillou]
13 ANGERS - Endocrino - Service d'Endocrinologie
14 Pathologie morphologique
15 Service de Chirurgie Digestive, Cancérologique, Générale, Endocrinienne et Urgences (CHU de Dijon)

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.

Mots clés: survival in MEN1 patients; br-NETs

Domaines: Pneumologie et système respiratoire

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⟩