壶腹周围癌（PAC）是一种较为少见的胃肠道恶性肿瘤，涵盖了Vater壶腹、胰头、胆总管下段和十二指肠乳头的恶性肿瘤。外科手术是该区域肿瘤的首选治疗方案，但部分患者无法手术或术后复发，化疗成为该疾病的重要辅助治疗手段。由于该区域组织来源种类多样、化疗方案纷杂、化疗效果尚不明确，同时国内、外临床指南亦缺乏统一、明确的建议。因此，化疗在PAC中的作用仍需进一步探究。通过对国内、外文献的查阅和临床相关治疗经验的总结，初步发现：（1）通过HE染色及免疫组化可以将PAC分为肠型、胰胆管型和混合型；（2）肠型患者选择以5-氟尿嘧啶为基础的化疗方案；胰胆管型和混合型患者选择以吉西他滨为基础的化疗方案可能会使患者更多受益。上述结论需要更为严谨的前瞻性随机对照试验来明确。因此，我们应重视组织病理分型，建议基于组织病理学分型来选择化疗方案；在临床研究中也应基于组织病理分型进行研究，这样能消除部分混杂因素，得出更为精确的结论。

Periampullary carcinoma is a relatively rare malignant gastrointestinal tumor, encompassing malignancies of the Vater's ampulla, pancreatic head, distal common bile duct, and duodenal papilla. The anatomy of this area is intricate, with a narrow spatial structure and complex surrounding relationships. When it comes to treating tumors in this area, surgery is the preferred option. However, in clinical practice, there are often situations where certain patients are unable to undergo surgery or experience postoperative recurrence due to various reasons. Consequently, the use of adjuvant treatment, particularly chemotherapy, has become an essential approach in managing periampullary cancer. The lack of unified and clear recommendations in domestic and foreign clinical guidelines is attributed to the diverse sources of tissue in this region, the complexity of chemotherapy regimens, and the unclear efficacy of chemotherapy. Therefore, the role of chemotherapy in periampullary carcinoma still needs to be further explored and studied. Through a comprehensive review of domestic and foreign literature and an analysis of clinically relevant treatment experiences, we have identified an important finding: (1) through HE staining and immunohistochemistry, it can be classified into intestinal type originating from intestinal tissues, pancreatic biliary type originating from pancreatic or bile duct tissues, and mixed type originating from both intestinal, pancreatic or bile duct tissues; (2) The chemotherapy regimen should be selected based on the tumor's histopathological origin, as choosing an appropriate regimen can improve the survival time of patients with periampullary carcinoma; (3) selection of 5-FU based on chemotherapy regimens for patients with intestinal-type (IN-type), selection of gemcitabine based on chemotherapy regimens may be more beneficial for patients with pancreatic biliary-type (PB-type) and ambiguous-type (AM-type). However, the final conclusion needs to be clarified by more rigorous prospective randomized controlled trials. Through a systematic analysis of research in this field, we believe that the traditional understanding of periampullary carcinoma should be changed in clinical work: firstly, in clinical work, we should collectively call the tumour in this region as periampullary carcinoma, instead of traditionally calling it duodenal papillary cancer, which will affect our judgement of the patient's prognosis and the choice of chemotherapy regimen. Secondly, we should discard the traditional concept that the prognosis of patients with periampullary carcinoma is better than that of pancreatic cancer and bile duct cancer. At present, the prognosis of patients with the IN-type may be better than that of patients with the PB-type; however, the prognosis of patients with the PB-type is not much different from that of patients with pancreatic and bile duct cancers. Thirdly, we need to be aware of histopathological typing, and it is recommended that chemotherapy regimens should be selected based on histopathological typing; studies should also be based on histopathological typing in clinical studies, which can eliminate some of the confounding factors and lead to more accurate conclusion.