Title |
Histologically Proven Bronchial Neuroendocrine Tumors in MEN1: A GTE 51‐Case Cohort Study
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Published in |
World Journal of Surgery, August 2017
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DOI | 10.1007/s00268-017-4135-z |
Pubmed ID | |
Authors |
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 |
Abstract |
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. 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. |
X Demographics
Geographical breakdown
Country | Count | As % |
---|---|---|
France | 1 | 50% |
Unknown | 1 | 50% |
Demographic breakdown
Type | Count | As % |
---|---|---|
Members of the public | 2 | 100% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
---|---|---|
Unknown | 15 | 100% |
Demographic breakdown
Readers by professional status | Count | As % |
---|---|---|
Student > Ph. D. Student | 4 | 27% |
Other | 3 | 20% |
Student > Doctoral Student | 1 | 7% |
Student > Master | 1 | 7% |
Researcher | 1 | 7% |
Other | 1 | 7% |
Unknown | 4 | 27% |
Readers by discipline | Count | As % |
---|---|---|
Medicine and Dentistry | 7 | 47% |
Nursing and Health Professions | 2 | 13% |
Unknown | 6 | 40% |