Biliary Neuroendocrine Neoplasms: Clinical Profiles, Management, and Analysis of Prognostic Factors

Biliary neuroendocrine neoplasms (NENs) represent <1% of all NENs. The aim of this retrospective study is to present the clinical characteristics, management and prognosis profiles of 28 biliary NEN patients from a large tertiary center, and identify factors related to prognosis. Nine tumors...

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Main Authors: Zhibo Zheng, Chuyan Chen, Binglu Li, Hongbo Liu, Liangrui Zhou, Hui Zhang, Chaoji Zheng, Xiaodong He, Wei Liu, Tao Hong, Yupei Zhao
Format: Article
Language:English
Published: Frontiers Media S.A. 2019-02-01
Series:Frontiers in Oncology
Subjects:
Online Access:https://www.frontiersin.org/article/10.3389/fonc.2019.00038/full
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author Zhibo Zheng
Chuyan Chen
Binglu Li
Hongbo Liu
Liangrui Zhou
Hui Zhang
Chaoji Zheng
Xiaodong He
Wei Liu
Tao Hong
Yupei Zhao
author_facet Zhibo Zheng
Chuyan Chen
Binglu Li
Hongbo Liu
Liangrui Zhou
Hui Zhang
Chaoji Zheng
Xiaodong He
Wei Liu
Tao Hong
Yupei Zhao
author_sort Zhibo Zheng
collection DOAJ
description Biliary neuroendocrine neoplasms (NENs) represent <1% of all NENs. The aim of this retrospective study is to present the clinical characteristics, management and prognosis profiles of 28 biliary NEN patients from a large tertiary center, and identify factors related to prognosis. Nine tumors originated from the gallbladder, two from the extrahepatic bile duct and 17 from the ampulla of Vater. One patient was classified as neuroendocrine tumor (NET) Grade 1, three patients were classified as NET Grade 2, 18 were graded neuroendocrine carcinoma (NEC) Grade 3 and six were classified as mixed adenoneuroendocrine carcinoma (MANEC). The overall survival rate and disease-free survival rate did not have statistically significant differences between tumors of different locations or different grading. Recurrence of disease correlated with poor prognosis (p < 0.001). Lymphovascular invasion and invasion beyond the submucosa were related to higher risk of local lymph node metastases. Multivariate analysis identified patient age (p = 0.021) and R0 resection margin (p = 0.027) as independent prognostic factors associated with overall survival. Our study included relatively large numbers of biliary tract NENs with intact follow-up information. Patients with biliary neuroendocrine tumors showed different clinical outcomes according to tumor locations and tumor grades. Achieving R0 resection is important for better prognosis.
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spelling doaj.art-e561356cb51b4beb9e52f6a1c9cd198c2022-12-22T01:17:51ZengFrontiers Media S.A.Frontiers in Oncology2234-943X2019-02-01910.3389/fonc.2019.00038431906Biliary Neuroendocrine Neoplasms: Clinical Profiles, Management, and Analysis of Prognostic FactorsZhibo Zheng0Chuyan Chen1Binglu Li2Hongbo Liu3Liangrui Zhou4Hui Zhang5Chaoji Zheng6Xiaodong He7Wei Liu8Tao Hong9Yupei Zhao10Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, ChinaDepartment of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, ChinaDepartment of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, ChinaSchool of Public Health, China Medical University, Shenyang, ChinaDepartment of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, ChinaDepartment of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, ChinaDepartment of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, ChinaDepartment of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, ChinaDepartment of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, ChinaDepartment of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, ChinaDepartment of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, ChinaBiliary neuroendocrine neoplasms (NENs) represent <1% of all NENs. The aim of this retrospective study is to present the clinical characteristics, management and prognosis profiles of 28 biliary NEN patients from a large tertiary center, and identify factors related to prognosis. Nine tumors originated from the gallbladder, two from the extrahepatic bile duct and 17 from the ampulla of Vater. One patient was classified as neuroendocrine tumor (NET) Grade 1, three patients were classified as NET Grade 2, 18 were graded neuroendocrine carcinoma (NEC) Grade 3 and six were classified as mixed adenoneuroendocrine carcinoma (MANEC). The overall survival rate and disease-free survival rate did not have statistically significant differences between tumors of different locations or different grading. Recurrence of disease correlated with poor prognosis (p < 0.001). Lymphovascular invasion and invasion beyond the submucosa were related to higher risk of local lymph node metastases. Multivariate analysis identified patient age (p = 0.021) and R0 resection margin (p = 0.027) as independent prognostic factors associated with overall survival. Our study included relatively large numbers of biliary tract NENs with intact follow-up information. Patients with biliary neuroendocrine tumors showed different clinical outcomes according to tumor locations and tumor grades. Achieving R0 resection is important for better prognosis.https://www.frontiersin.org/article/10.3389/fonc.2019.00038/fullneuroendocrine neoplasmgallbladderbiliary tractampullasurvival
spellingShingle Zhibo Zheng
Chuyan Chen
Binglu Li
Hongbo Liu
Liangrui Zhou
Hui Zhang
Chaoji Zheng
Xiaodong He
Wei Liu
Tao Hong
Yupei Zhao
Biliary Neuroendocrine Neoplasms: Clinical Profiles, Management, and Analysis of Prognostic Factors
Frontiers in Oncology
neuroendocrine neoplasm
gallbladder
biliary tract
ampulla
survival
title Biliary Neuroendocrine Neoplasms: Clinical Profiles, Management, and Analysis of Prognostic Factors
title_full Biliary Neuroendocrine Neoplasms: Clinical Profiles, Management, and Analysis of Prognostic Factors
title_fullStr Biliary Neuroendocrine Neoplasms: Clinical Profiles, Management, and Analysis of Prognostic Factors
title_full_unstemmed Biliary Neuroendocrine Neoplasms: Clinical Profiles, Management, and Analysis of Prognostic Factors
title_short Biliary Neuroendocrine Neoplasms: Clinical Profiles, Management, and Analysis of Prognostic Factors
title_sort biliary neuroendocrine neoplasms clinical profiles management and analysis of prognostic factors
topic neuroendocrine neoplasm
gallbladder
biliary tract
ampulla
survival
url https://www.frontiersin.org/article/10.3389/fonc.2019.00038/full
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