Cai Xing-Chen, Wu Sheng-Dong
Department of Hepato-Pancreato-Billiary Surgery, The affiliated Lihuili Hospital, Ningbo University, Ningbo, Zhejiang, China.
World J Clin Cases. 2022 Aug 16;10(23):8212-8223. doi: 10.12998/wjcc.v10.i23.8212.
Gallbladder neuroendocrine carcinoma (GB-NEC) has a low incidence rate; therefore, its clinical characteristics, diagnosis, treatment and prognosis are not well explored.
To review recent research and analyze corresponding data in the Surveillance Epidemiology and End Results (SEER) database.
Data of GB-NEC ( = 287) and gallbladder adenocarcinoma (GB-ADC) ( = 19 484) patients from 1975 to 2016 were extracted from the SEER database. Survival analysis was performed using Kaplan-Meier and Cox proportional hazards regression. < 0.05 was considered statistically significant. We also reviewed 108 studies retrieved from PubMed and (https://www.referencecitationanalysis.com/). The keywords used for the search were: "(Carcinoma, Neuroendocrine) AND (Gallbladder Neoplasms)".
The GB-NEC incidence rate was 1.6% (of all gallbladder carcinomas), male to female ratio was 1:2 and the median survival time was 7 mo. The 1-, 2-, 3- and 5-year overall survival (OS) was 36.6%, 17.8%, 13.2% and 7.3% respectively. Serum chromogranin A levels may be a specific tumor marker for the diagnosis of GB-NEC. Elevated carcinoembryonic antigen, carbohydrate antigen (CA)-19-9 and CA-125 levels were associated with poor prognosis. Age [hazard ratio (HR) = 1.027, 95% confidence interval (CI): 1.006-1.047, = 0.01] and liver metastasis (HR = 3.055, 95% CI: 1.839-5.075, < 0.001) are independent prognostic risk factors for OS. Patients with advanced GB-NEC treated with surgical resection combined with radiotherapy and/or chemotherapy may have a better prognosis than those treated with surgical resection alone. There was no significant difference in OS between GB-NEC and GB-ADC.
The clinical manifestations and prognosis of GB-NEC are similar to GB-ADC, but the treatment is completely different. Early diagnosis and treatment are the top priorities.
胆囊神经内分泌癌（GB-NEC）发病率较低；因此，其临床特征、诊断、治疗及预后尚未得到充分研究。
回顾近期研究并分析监测、流行病学与最终结果（SEER）数据库中的相应数据。
从SEER数据库中提取1975年至2016年GB-NEC患者（n = 287）和胆囊腺癌（GB-ADC）患者（n = 19484）的数据。采用Kaplan-Meier法和Cox比例风险回归进行生存分析。P < 0.05被认为具有统计学意义。我们还回顾了从PubMed和（https://www.referencecitationanalysis.com/）检索到的108项研究。搜索所用关键词为：“（癌，神经内分泌）AND（胆囊肿瘤）”。
GB-NEC发病率为所有胆囊癌的1.6%，男女比例为1:2，中位生存时间为7个月。1年、2年、3年和5年总生存率（OS）分别为36.6%、17.8%、13.2%和7.3%。血清嗜铬粒蛋白A水平可能是诊断GB-NEC的特异性肿瘤标志物。癌胚抗原、糖类抗原（CA）-19-9和CA-125水平升高与预后不良相关。年龄[风险比（HR） = 1.027，95%置信区间（CI）：1.006 - 1.047，P = 0.01]和肝转移（HR = 3.055，95%CI：1.839 - 5.075，P < 0.001）是OS的独立预后危险因素。接受手术切除联合放疗和/或化疗的晚期GB-NEC患者可能比仅接受手术切除的患者预后更好。GB-NEC和GB-ADC的OS无显著差异。
GB-NEC的临床表现和预后与GB-ADC相似，但治疗完全不同。早期诊断和治疗是首要任务。
