Department of Hepatobiliary and Pancreatic Oncology, National Cancer Center Hospital, 5-1-1, Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.
Department of Hepatobiliary and Pancreatic Oncology, National Cancer Center Hospital East, Chiba, Japan.
J Gastroenterol. 2022 Nov;57(11):890-901. doi: 10.1007/s00535-022-01920-5. Epub 2022 Sep 26.
Information on rare hepatobiliary and pancreatic (HBP) subtypes of cancer is scarce. We aimed to elucidate the incidence and clinical features of rare tumors in Japan using the National Cancer Registry (NCR), a new nationwide integrated population-based registry.
The data of patients diagnosed in 2016-2017 were extracted from the NCR database, and classified by topography: liver cells, intrahepatic bile duct, gallbladder, extrahepatic bile duct, ampulla of Vater, and pancreas. Data were described and analyzed using the World Health Organization and General Rules tumor classifications. The incidences for all rare tumors including hepatoblastoma and adenosquamous cell carcinoma were calculated as the number of new cases divided by the corresponding total person years.
The NCR data yielded 8,239 patients with rare HBP tumors between 2016 and 2017. The ratios of rare tumors to all cancer types were 0.5%, 0.7%, 3.9%, 1.6%, 0.8%, and 7.2% in the liver, intrahepatic bile duct, gallbladder, extrahepatic bile duct, ampulla of Vater, and pancreas, respectively. Rare tumors occurred more frequently in men, except for gallbladder tumors. The main tumor stage was localized in liver cells (42.4%) and the intrahepatic bile duct (51.6%); more patients were diagnosed in advanced stage with gallbladder (84.1%) and extrahepatic bile duct (74.4%) tumors. Approximately equal percentage of patients were diagnosed at designated cancer care hospitals (DCCHs) and non-DCCHs, whereas 60% to 70% patients received treatment at DCCHs.
This is the first report to provide comprehensive information on the epidemiological status of rare HBP tumors in Japan by utilizing population-based NCR data.
有关罕见肝胆胰（HBP）肿瘤亚型的信息很少。我们旨在利用新的全国性综合人群登记处——国家癌症登记处（NCR）阐明日本罕见肿瘤的发病率和临床特征。
从 NCR 数据库中提取了 2016-2017 年诊断的患者数据，并按部位分类：肝细胞、肝内胆管、胆囊、肝外胆管、壶腹和胰腺。使用世界卫生组织和一般规则肿瘤分类对数据进行描述和分析。将所有罕见肿瘤（包括肝母细胞瘤和腺鳞癌）的发病率计算为新病例数除以相应的总人数年。
NCR 数据在 2016 年至 2017 年间提供了 8239 例罕见 HBP 肿瘤患者。罕见肿瘤占所有癌症类型的比例分别为肝脏、肝内胆管、胆囊、肝外胆管、壶腹和胰腺的 0.5%、0.7%、3.9%、1.6%、0.8%和 7.2%。除了胆囊肿瘤外，罕见肿瘤更常见于男性。主要肿瘤分期为局限性在肝细胞（42.4%）和肝内胆管（51.6%）；更多的胆囊（84.1%）和肝外胆管（74.4%）肿瘤患者在晚期诊断。大约相同比例的患者在指定癌症治疗医院（DCCH）和非 DCCH 诊断，而 60%至 70%的患者在 DCCH 接受治疗。
这是首次利用基于人群的 NCR 数据提供日本罕见 HBP 肿瘤的流行病学综合信息的报告。
Zotero 插件
