壶腹或乳头癌是一种恶性肿瘤，起源于主十二指肠乳头的粘膜区域，也被称为瓦特壶腹。无论在辅助治疗情况下还是在姑息疗法中，目前均缺乏统一的处理建议。我们在PubMed上进行了有选择性的文献搜索，以确定关于该病病因流行病学、临床病理背景、外科和药物治疗的最有信息量的出版物。壶腹癌的发病率为每10万人口0.5至0.9人，并且预后差，局部受限的5年生存率为41%至45%，而转移性疾病的为4%至7%。大多数此类肿瘤是肠道或胰胆同源的免疫组化亚型；后者的预后较差（中位生存期为72-80个月，而前者为33-41个月）。目前还没有针对任何亚型的靶向治疗，并且在辅助治疗或姑息治疗情况下，尚未有足够的科学证据来制定特定的治疗建议。壶腹癌的首选治疗是胰头根治性切除术，并进行系统性淋巴结清除术。根据病情不同，5年总生存率为10%至75%不等。对辅助治疗没有明确的建议。姑息治疗可以参考癌症结肠、胰腺和胆管的已发表治疗策略。当前壶腹癌治疗的证据状况较差。治疗决策应在跨学科肿瘤委员会中进行讨论，并应根据组织学亚型考虑。

Ampullary or papillary carcinoma is a malignant tumor arising from the mucosa in the region of the major duodenal papilla, also known as the ampulla of Vater. Uniform treatment recommendations are lacking both for the adjuvant situation and for palliative care.A selective literature search was carried out in PubMed in order to identify the most informative publications concerning the epidemiology, clinico-pathological background, and surgical and medical treatment of this condition.Ampullary carcinoma has an incidence of 0.5 to 0.9 per 100 000 persons and a poor prognosis, with a 5-year survival rate of 41% to 45% for locally confined and 4% to 7% for metastatic disease. Most such tumors are of an intestinal or a pancreaticobiliary immunohistochemical subtype; the latter has a worse prognosis (median survival, 72-80 vs. 33-41 months). Targeted treatment is not yet available for either subtype, nor is there enough scientific evidence available for the formulation of specific therapeutic recommendations in either the adjuvant or the palliative situation. The treatment of choice for ampullary carcinoma is radical oncological resection of the head of the pancreas with systematic lymphadenectomy. Five-year overall survival is less than 10% to 75% depending on the stage. No definitive recommendation for adjuvant therapy can be given. Palliative therapy can be oriented to the published treatment strategies for cancer of the colon, pancreas, and bile duct.The current state of the evidence on the treatment of ampullary carcinoma is poor. Therapeutic decisions should be discussed in an interdisciplinary tumor board and should, in our opinion, take the histological subtype into account.