Dataset Information

The clinical profiles, management, and prognostic factors of biliary mixed neuroendocrine nonneuroendocrine neoplasms: A systematic review of the literature.

ABSTRACT:

Background

Mixed neuroendocrine nonneuroendocrine neoplasms (MiNENs) originating from the biliary system (gallbladder, biliary tract, or ampulla of Vater) are extremely rare and have not been discussed in detail or systematically. We aimed to present the demographics, clinicopathological characteristics, management, and prognostic factors of biliary MiNENs.

Methods

A systematic search of electronic biomedical databases (Web of Science, PUBMED, and Embase) was performed to identify eligible studies. Survival was analyzed with the Kaplan-Meier method. Log-rank tests were used to evaluate the differences between groups, and the effects of various clinical and histopathological features on prognosis were analyzed by univariate and multivariate Cox regression.

Results

Fifty-three publications (patients, n?=?67) were included. The median overall survival time was 21.0 months. Fifty-one patients (76.1%) underwent radical surgery and median survival for 41 months (P?ConclusionRadical resection is essential for long-term survival. Aggressive multimodality therapy with adjuvant radiochemotherapy and biotherapy may improve survival of biliary MiNENs. Further randomized controlled trials are needed to determine the standard treatment.

SUBMITTER: Wen LJ

PROVIDER: S-EPMC7738038 | biostudies-literature | 2020 Dec

REPOSITORIES: biostudies-literature

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Publications

The clinical profiles, management, and prognostic factors of biliary mixed neuroendocrine nonneuroendocrine neoplasms: A systematic review of the literature.

Wen Li-Jia LJ Chen Jun-Hong JH Xu Hong-Ji HJ Yu Qiong Q Deng Yu Y Liu Kai K Liu Kai K

Medicine 20201201 50

<h4>Background</h4>Mixed neuroendocrine nonneuroendocrine neoplasms (MiNENs) originating from the biliary system (gallbladder, biliary tract, or ampulla of Vater) are extremely rare and have not been discussed in detail or systematically. We aimed to present the demographics, clinicopathological characteristics, management, and prognostic factors of biliary MiNENs.<h4>Methods</h4>A systematic search of electronic biomedical databases (Web of Science, PUBMED, and Embase) was performed to identify ...[more]

PMID: 33327249

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Project description:IntroductionMixed neuroendocrine non-neuroendocrine neoplasm (MiNEN) is a rare form of neuroendocrine neoplasms (NENs). The purpose of this study was to investigate the characteristics and survival profile of appendiceal MiNENs, with a view of providing robust clinical features of this rare disease.MethodsPatients were selected from the Surveillance, Epidemiology, and End Results database (2004-2016). The prognosis of MiNEN (n = 315) was compared with other histological subtypes including neuroendocrine tumor (NETs) (n = 1734), neuroendocrine carcinoma (NECs) (n = 375), goblet cell carcinoid (GCC) (n = 968), signet ring cell carcinoma (n = 463), mucinous adenocarcinoma (MAC) (n = 2355), and non-mucinous adenocarcinoma (NMAC) (n = 1187) in the appendix. Age-adjusted incidence was calculated using Joinpoint regression. The Cox proportional hazards model and the Fine-Gray competing risk model were used to perform overall survival (OS) and cancer-specific mortality (CSM) analyses, respectively.ResultsThe age-adjusted incidence of MiNENs increased from 0.01/100,000 person-years in 2004 to 0.07/100,000 person-years in 2016. The 3-, 5-, and 10-year OS rates for MiNENs were 69.5, 57.4, and 43.7%, respectively, and the corresponding CSM rates were 23.1, 36.4, and 45.1%, respectively. Multivariate analysis revealed that the prognosis of MiNENs was worse than that of NETs, NECs, GCC, and MAC but better than that of NMAC and signet ring cell carcinoma. Tumor extension was the only independent factor influencing the prognosis of MiNENs, but tumor size, grade, and surgical approaches were not. Moreover, when compared with local excision or appendectomy, extensive surgery such as hemicolectomy or colectomy did not prolong the survival of individuals with MiNENs.ConclusionMiNEN is a rare but aggressive tumor with a poor prognosis differing from NENs, GCC and adenocarcinomas. To improve the prognosis of the disease, early diagnosis and comprehensive evaluation are necessary.

Dataset Information

The clinical profiles, management, and prognostic factors of biliary mixed neuroendocrine nonneuroendocrine neoplasms: A systematic review of the literature.

Background

Methods

Results

Publications

The clinical profiles, management, and prognostic factors of biliary mixed neuroendocrine nonneuroendocrine neoplasms: A systematic review of the literature.

Similar Datasets

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