Department of Biliary Surgery, West China Hospital, Sichuan University, Chengdu, China; Department of Biliary Disease Research Center, West China Hospital, Sichuan University, Chengdu, China.
Biomol Biomed. 2023 Nov 3;23(6):1096-1107. doi: 10.17305/bb.2023.9582.
Gallbladder neuroendocrine carcinoma (GBNEC) is rare and characterized by a low degree of tumor differentiation. The clinical features of GBNEC versus gallbladder adenocarcinoma (GBADC) remain a subject of debate. A total of 201 GBADC and 36 GBNEC cases that underwent surgery resection between January 2010 and 2022 at the Department of Biliary Surgery, West China Hospital, Sichuan University were included. A 1:1 propensity score matching (PSM) was performed based on seven predefined variables: age, sex, the American Joint Committee on Cancer (AJCC) stage, resection status, perineural invasion (PNI), lymphovascular invasion (LVI), and degree of tumor differentiation. Compared with GBADC, GBNEC patients were younger (median age 56.0 vs 64.0 years; P = 0.001), and more patients presented with advanced stages of tumor (P = 0.003). Patients with GBNEC also had a higher rate of PNI (55.6% vs 22.4%; P < 0.001), and LVI (63.9% vs 45.80%; P = 0.658). Before PSM, GBNEC patients had inferior prognoses compared with GBADC patients with a shorter median overall survival (mOS) (15.02 vs 20.11 months; P = 0.0028) and a shorter median recurrence-free survival (mRFS) (10.30 vs 15.17 months; P = 0.0028). However, after PSM analyses, there were no differences in OS (mOS 18.6 vs 18.0 months; P=0.24) or RFS (mRFS 10.98 vs 12.02 months; P = 0.39) between the GBNEC and GBADC cases. After multivariate analysis, tumor diagnosis (GBNEC vs GBADC) was not identified as an independent risk factor for shorter RFS (P = 0.506) or OS (P = 0.731). Unfavorable pathological features, including advanced AJCC tumor stages, poor differentiation, presence of LVI, and positive resection margins (all P < 0.05), were independent risk factors for inferior OS and RFS. GBNEC is difficult to diagnose early and has a prognosis comparable to stage-matched poorly differentiated GBADC. Tumor diagnosis (either GBADC or GBNEC) was not an independent risk factor for the patient's OS. Unfavorable pathological features of the neoplasm are the main determinants.
胆囊神经内分泌癌（GBNEC）较为罕见，其肿瘤分化程度较低。GBNEC 与胆囊腺癌（GBADC）的临床特征仍存在争议。本研究回顾性分析了 2010 年 1 月至 2022 年期间在四川大学华西医院胆道外科接受手术切除的 201 例 GBADC 和 36 例 GBNEC 患者的临床病理资料。根据年龄、性别、美国癌症联合委员会（AJCC）分期、手术切除状态、神经周围侵犯（PNI）、脉管侵犯（LVI）和肿瘤分化程度等 7 个预设变量进行 1:1 倾向评分匹配（PSM）。与 GBADC 相比，GBNEC 患者年龄更小（中位年龄 56.0 岁 vs 64.0 岁；P = 0.001），且更多患者处于肿瘤晚期（P = 0.003）。GBNEC 患者的 PNI 发生率（55.6% vs 22.4%；P < 0.001）和 LVI 发生率（63.9% vs 45.80%；P = 0.658）更高。在 PSM 之前，与 GBADC 患者相比，GBNEC 患者的预后较差，总生存（OS）时间更短（中位 OS 15.02 个月 vs 20.11 个月；P = 0.0028），无复发生存（RFS）时间更短（中位 RFS 10.30 个月 vs 15.17 个月；P = 0.0028）。然而，PSM 分析后，GBNEC 和 GBADC 患者的 OS（mOS 18.6 个月 vs 18.0 个月；P=0.24）或 RFS（mRFS 10.98 个月 vs 12.02 个月；P = 0.39）均无差异。多因素分析显示，肿瘤诊断（GBNEC 与 GBADC）不是 RFS（P = 0.506）或 OS（P = 0.731）较短的独立危险因素。不良的病理特征，包括 AJCC 肿瘤分期较晚、分化程度差、LVI 存在和阳性切缘（均 P < 0.05），是 OS 和 RFS 较差的独立危险因素。GBNEC 早期诊断困难，预后与匹配的 AJCC 分期较差的分化型 GBADC 相当。肿瘤诊断（GBADC 或 GBNEC）不是患者 OS 的独立危险因素。肿瘤的不良病理特征是主要决定因素。
