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      Clinicopathological features and survival for gallbladder NEN: a population-based study


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          Gallbladder neuroendocrine neoplasm (GB-NEN) is a relatively rare neoplasm, accounting for 0.5% of all neuroendocrine neoplasm cases and 2.1% of gallbladder cancers. Because of the limited understanding of GB-NEN, the aim of this study was to explore the clinicopathology and survival of GB-NEN patients selected from the Surveillance, Epidemiology, and End Results (SEER) database.


          A total of 248 GB-NEN patients from the SEER database diagnosed between 2004 and 2015 were included. Kaplan–Meier curves were used to examine the survival time. Multivariate Cox proportional hazard models were used to estimate hazard ratios with 95% confidence intervals to analyze the impact of factors on overall survival and cancer-specific survival.


          The majority of the GB-NEN patients were women (67.3%), white (77%), and married (61.7%). Most tumors were <2 cm in size (31.0%), G3 stage (25.8%), and distant SEER stage (41.1%). 62.9% and 64.5% of cases showed an absence of lymph node metastasis and tumor metastasis, respectively. Patients who received gallbladder surgery had significantly better survival outcomes ( P < 0.001). However, patients who received both gallbladder surgery and lymph node resection did not have better survival outcome compared with patients who received only gallbladder surgery. Multivariate Cox proportional hazard models indicated that older age, unmarried status, large tumor size (>5 cm), and distant SEER stage were significant independent predictors for decreased overall survival time and cancer-specific survival time ( P < 0.05).


          Age, marital status, tumor size, and SEER stage were predictors for the survival of GB-NEN patients. Gallbladder surgery was associated with better survival, but the combination of gallbladder surgery and lymphadenectomy had no effect on survival outcomes.

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          A common classification framework for neuroendocrine neoplasms: an International Agency for Research on Cancer (IARC) and World Health Organization (WHO) expert consensus proposal

          The classification of neuroendocrine neoplasms (NENs) differs between organ systems and currently causes considerable confusion. A uniform classification framework for NENs at any anatomical location may reduce inconsistencies and contradictions among the various systems currently in use. The classification suggested here is intended to allow pathologists and clinicians to manage their patients with NENs consistently, while acknowledging organ-specific differences in classification criteria, tumor biology, and prognostic factors. The classification suggested is based on a consensus conference held at the International Agency for Research on Cancer (IARC) in November 2017 and subsequent discussion with additional experts. The key feature of the new classification is a distinction between differentiated neuroendocrine tumors (NETs), also designated carcinoid tumors in some systems, and poorly differentiated NECs, as they both share common expression of neuroendocrine markers. This dichotomous morphological subdivision into NETs and NECs is supported by genetic evidence at specific anatomic sites as well as clinical, epidemiologic, histologic, and prognostic differences. In many organ systems, NETs are graded as G1, G2, or G3 based on mitotic count and/or Ki-67 labeling index, and/or the presence of necrosis; NECs are considered high grade by definition. We believe this conceptual approach can form the basis for the next generation of NEN classifications and will allow more consistent taxonomy to understand how neoplasms from different organ systems inter-relate clinically and genetically.
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            Neuroendocrine tumors of the gallbladder: an evaluation and reassessment of management strategy.

            Gallbladder neuroendocrine tumors (GB-NETs) represent only 0.5% of all NETs, and little is known about their biological behavior. We sought to provide an overview of the current state of knowledge about GB-NETs and provide a recommendation for management. A PubMed search was undertaken using the following criteria: primary gallbladder and carcinoid or NET. We also interrogated the SEER 9, 13 and 17 registries (1973 to 2005) and the Niigata registry (2003) to assess the epidemiology and clinicopathological characteristics. Finally, we compared the clinical presentation, management and prognosis of GB-NETs to that of gallbladder adenocarcinoma. GB-NETs probably derive from either a multipotent stem cell or neuroendocrine cells in intestinal or gastric metaplasia of the gallbladder epithelium, which occurs consequent upon cholelithiasis/chronic inflammation. Clinically and at surgery, GB-NETs are indistinguishable from gallbladder cancer (GBC) and "carcinoid syndrome" is evident in only ∼1%.The median survival was only 9.8 months among 278 cases of GB-NETs reported in SEER. The 5 year survival rate for tumors classified as carcinoids/neuroendocrine carcinoma or small cell cancer (SCC) was 36.9 and 0%, respectively. Soga divided GB-NETs into typical and variant carcinoids with 5 year survival rates of 60.4% and 21.3% respectively. GB-NETs have an aggressive behavior, and once diagnosed, extensive surgical management and careful NET follow up with CT scan is mandatory to facilitate early detection of recurrence. Since more aggressive surgical management for GBC has shown increased survival rates for these tumors, a similar strategy seems reasonable for GB-NETs. However, in high grade metastatic tumors, the primary management is mainly medical.
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              An aggressive surgical approach leads to improved survival in patients with gallbladder cancer: a 12-year study at a North American Center.

              To determine if an aggressive surgical approach, with an increase in R0 resections, has resulted in improved survival for patients with gallbladder cancer. Many physicians express a relatively nihilistic approach to the treatment of gallbladder cancer; consensus among surgeons regarding the indications for a radical surgical approach has not been reached. A retrospective review of all patients with gallbladder cancer admitted during the past 12 years was conducted. Ninety-nine patients were identified. Cases treated during the 12-year period 1990 to 2002 were divided into 2 time-period (TP) cohorts, those treated in the first 6 years (TP1, N = 35) and those treated in the last 6 years (TP2, N = 64). Disease stratification by stage and other demographic features were similar in the 2 time periods. An operation with curative intent was performed on 38 patients. Nine (26%) R0 resections were performed in TP1 and 24 (38%) in TP2. The number of liver resections, as well as the frequency of extrahepatic biliary resections, was greater in TP2 (P < 0.04). In both time periods, an R0 resection was associated with improved survival (P < 0.02 TP1, P < 0.0001 TP2). Overall survival of all patients in TP2 was significantly greater than in TP1 (P < 0.03), with a median survival of 9 months in TP1 and 17 months in TP2. The median 5-year survival in TP1 was 7%, and 35% in TP2. The surgical mortality rate for the entire cohort was 2%, with a 49% morbidity rate. A margin-negative, R0 resection leads to improved survival in patients with gallbladder cancer.

                Author and article information

                Endocr Connect
                Endocr Connect
                Endocrine Connections
                Bioscientifica Ltd (Bristol )
                September 2019
                08 August 2019
                : 8
                : 9
                : 1273-1281
                [1 ]Key Laboratory of Laparoscopic Technology of Zhejiang Province , Sir Run-Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou, China
                [2 ]Department of General Surgery , Sir Run-Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou, China
                [3 ]Department of Internal Medicine , John H Stroger Hospital of Cook County, Chicago, Illinois, USA
                Author notes
                Correspondence should be addressed to J Xu or Y Liang: walter235@ 123456zju.edu.cn or 3312019@ 123456zju.edu.cn
                © 2019 The authors

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

                : 17 July 2019
                : 08 August 2019

                gallbladder neuroendocrine neoplasm,clinicopathological characteristics,survival,seer database


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