TY - JOUR
T1 - Histologically Proven Bronchial Neuroendocrine Tumors in MEN1
T2 - A GTE 51-Case Cohort Study
AU - Lecomte, P.
AU - Binquet, C.
AU - Le Bras, M.
AU - Tabarin, A.
AU - Cardot-Bauters, C.
AU - Borson-Chazot, F.
AU - Lombard-Bohas, C.
AU - Baudin, E.
AU - Delemer, B.
AU - Klein, M.
AU - Vergès, B.
AU - Aparicio, T.
AU - Cosson, E.
AU - Beckers, A.
AU - Caron, Ph
AU - Chabre, O.
AU - Chanson, Ph
AU - Du Boullay, H.
AU - Guilhem, I.
AU - Niccoli, P.
AU - Rohmer, V.
AU - Guigay, J.
AU - Vulpoi, C.
AU - Scoazec, J. Y.
AU - Goudet, P.
N1 - Publisher Copyright:
© 2017, Société Internationale de Chirurgie.
PY - 2018/1/1
Y1 - 2018/1/1
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=85026904462&partnerID=8YFLogxK
U2 - 10.1007/s00268-017-4135-z
DO - 10.1007/s00268-017-4135-z
M3 - Article
C2 - 28785839
AN - SCOPUS:85026904462
SN - 0364-2313
VL - 42
SP - 143
EP - 152
JO - World Journal of Surgery
JF - World Journal of Surgery
IS - 1
ER -