文献中不同壶腹周围癌（胰腺导管腺癌、远端胆管癌、壶腹癌和十二指肠癌）的患病率存在​​差异。 2010 年代，瑞典采用了基于轴向切片的胰十二指肠切除标本标准化组织病理学方案。本研究旨在提供有关壶腹周围癌的信息，包括治疗性和非治疗性环境中的肿瘤类型、术前诊断准确性以及手术标本标准化评估对诊断、R 状态和淋巴结评估的影响。来自诊断为壶腹周围癌的患者的数据从瑞典国家胰腺和壶腹周围癌登记处检索了 2010 年至 2019 年的壶腹周围癌。在非治愈患者中，3704 名（83.6%）被诊断为胰腺导管腺癌。在接受胰十二指肠切除术治疗的患者中，诊断为胰腺导管腺癌 1380 例（50.0%），远端胆管癌 284 例（10.3%），壶腹癌 376 例（13.6%），十二指肠癌 160 例（5.8%），其他560 名（20.3%）患者得到诊断。胰腺导管腺癌术前诊断与术后诊断相符，胰腺导管腺癌患者术前诊断与术后诊断相符，远端胆管癌患者术前诊断与术后诊断一致，远端胆管癌患者术前诊断与术后诊断一致，壶腹癌患者术前诊断与术后诊断一致，壶腹癌患者术前诊断与术后诊断一致，为120例（53.6%）。十二指肠癌。在接受标准化评估的手术标本中，胰腺导管腺癌的发生率较高，从 56.8% 升至 64.3%（P = 0.003）。标准化后，R1切除率（31.7% vs 44.6%，P < 0.001）和N1期切除率（62.1% vs 77.0%，P < 0.001）较高。患者中胰腺导管腺癌的比例较高在非治愈性环境中与接受手术的患者进行比较。壶腹周围癌的误诊率被证实很高。因此，在考虑术前肿瘤治疗时应考虑到这一点。手术标本的标准化评估增加了胰腺导管腺癌、R1 和 N1 率。© 作者 2023。由牛津大学出版社代表 BJS Society Ltd 出版。

The prevalence of different periampullary cancers (pancreatic ductal adenocarcinoma, distal cholangiocarcinoma, ampullary cancer and duodenal cancer) is heterogeneous in the literature. During the 2010s, a standardized histopathological protocol for pancreatoduodenectomy specimens based on axial slicing was adopted in Sweden. The present study sought to provide information about periampullary cancers with regard to tumour types in curative and noncurative settings, preoperative diagnostic accuracy and the impact of a standardized evaluation of the surgical specimen on diagnosis, R status and lymph node assessment.Data from patients diagnosed with periampullary cancer from 2010 to 2019 were retrieved from the Swedish National Registry for Pancreatic and Periampullary Cancer.Among non-curative patients, 3704 (83.6 per cent) were diagnosed with pancreatic ductal adenocarcinoma. Among patients treated with pancreatoduodenectomy, diagnosis was pancreatic ductal adenocarcinoma in 1380 (50.0 per cent), distal cholangiocarcinoma in 284 (10.3 per cent), ampullary cancer in 376 (13.6 per cent), duodenal cancer in 160 (5.8 per cent) and other diagnoses in 560 (20.3 per cent) patients. The preoperative diagnosis corresponded to the postoperative in 1177 (67.5 per cent) patients for pancreatic ductal adenocarcinoma, 162 (37.4 per cent) patients for distal cholangiocarcinoma, 220 (61.3 per cent) patients for ampullary cancer and 120 (53.6 per cent) patients for duodenal cancer. A higher rate of pancreatic ductal adenocarcinoma was seen in surgical specimens who underwent standardized evaluation, from 56.8 per cent to 64.3 per cent (P = 0.003). After standardization, higher rates of R1 resection (31.7 per cent versus 44.6 per cent, P < 0.001) and N1 stage (62.1 per cent versus 77.0 per cent, P < 0.001) were found.The proportion of pancreatic ductal adenocarcinoma was higher in patients in a non-curative setting compared with patients who underwent surgery. The rate of misdiagnosis for periampullary cancers was confirmed to be high. Thus, it should be taken into account when preoperative oncological treatment is considered. Standardized evaluation of the surgical specimen has increased pancreatic ductal adenocarcinoma, R1 and N1 rates.© The Author(s) 2023. Published by Oxford University Press on behalf of BJS Society Ltd.