Division of Hepatobiliary Pancreatic Surgery, First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310003, China.
Division of Hepatobiliary Pancreatic Surgery, Shulan (Hangzhou) Hospital, Hangzhou 310004, China.
Hepatobiliary Pancreat Dis Int. 2019 Dec;18(6):538-545. doi: 10.1016/j.hbpd.2019.03.009. Epub 2019 Apr 5.
Primary hepatic neuroendocrine neoplasms (PHNENs) are extremely rare and few articles have compared the prognosis of PHNENs with other neuroendocrine neoplasms (NENs). This study aimed to investigate the different prognosis between PHNENs and pancreatic NEN (PanNENs) and evaluate the relevant prognosis-related factors.
From January 2012 to October 2016, a total of 44 NENs patients were enrolled and divided into two groups according to the primary tumor location which were named group PHNENs (liver; n = 12) and group PanNENs (pancreas; n = 32). Demographic, clinical characteristics and survival data were compared between the two groups with Kaplan-Meier method and log-rank tests. Prognostic factors were analyzed using the Cox regression model.
The overall survival of group PHNENs and group PanNENs were 25.4 ± 6.7 months and 39.8 ± 3.7 months, respectively (P = 0.037). The cumulative survival of group PanNENs was significantly higher than that of group PHNENs (P = 0.029). Univariate analysis revealed that sex, albumin, total bilirubin, total bile acid, aspartate aminotransferase, alkaline phosphatase, α-fetoprotein and carbohydrate antigen 19-9, histological types, treatments and primary tumor site were the prognostic factors. Further multivariate analysis indicated that albumin (P = 0.008), histological types NEC (P = 0.035) and treatments (P = 0.005) were the independent prognostic factors. Based on the histological types, the cumulative survival of patients with well-differentiated neuroendocrine tumor was significant higher than that of patients with poorly differentiated neuroendocrine carcinoma in group PHNENs (P = 0.022), but not in group PanNENs (P > 0.05). According to the different treatments, patients who received surgery had significantly higher cumulative survival than those with conservative treatment in both groups (P < 0.05).
PHNENs have lower survival compared to PanNENs. Histological types and treatments affect the prognosis. Surgical resection still remains the first line of treatment for resectable lesions and can significantly improve the survival.
原发性肝神经内分泌肿瘤（PHNENs）极为罕见，鲜有文献比较 PHNENs 与其他神经内分泌肿瘤（NENs）的预后。本研究旨在探讨 PHNENs 与胰腺神经内分泌肿瘤（PanNENs）之间的不同预后，并评估相关预后因素。
2012 年 1 月至 2016 年 10 月，共纳入 44 例 NENs 患者，根据原发肿瘤部位分为两组，分别命名为 PHNENs 组（肝脏；n=12）和 PanNENs 组（胰腺；n=32）。采用 Kaplan-Meier 法和对数秩检验比较两组患者的人口统计学、临床特征和生存数据。采用 Cox 回归模型分析预后因素。
PHNENs 组和 PanNENs 组的总生存率分别为 25.4±6.7 个月和 39.8±3.7 个月（P=0.037）。PanNENs 组的累积生存率明显高于 PHNENs 组（P=0.029）。单因素分析显示，性别、白蛋白、总胆红素、总胆汁酸、天门冬氨酸氨基转移酶、碱性磷酸酶、甲胎蛋白和癌胚抗原 19-9、组织学类型、治疗方法和原发肿瘤部位是预后因素。进一步多因素分析表明，白蛋白（P=0.008）、组织学类型 NEC（P=0.035）和治疗方法（P=0.005）是独立的预后因素。根据组织学类型，PHNENs 组中分化良好的神经内分泌肿瘤患者的累积生存率明显高于低分化神经内分泌癌患者（P=0.022），但在 PanNENs 组中无差异（P＞0.05）。根据不同的治疗方法，两组中接受手术治疗的患者的累积生存率明显高于保守治疗的患者（P＜0.05）。
PHNENs 的生存率低于 PanNENs。组织学类型和治疗方法影响预后。手术切除仍然是可切除病变的一线治疗方法，可以显著提高生存率。
