Abstract
Extrahepatic cholangiocarcinoma (EHCC) is a rare malignant tumor, and current treatment methods are also relatively limited. Radical surgery is the only potentially curative method for the long survival time. However, despite undergoing radical resection, prognosis remained poor due to the high recurrence rate and distant metastasis. Therefore, adjuvant chemotherapy and radiotherapy should be offered to patients who have undergone surgery. Unfortunately, the low incidence of this disease has resulted in a lack of high-level evidence to confirm the importance of adjuvant chemotherapy or radiotherapy. At present, it is still controversial whether adjuvant therapy can prolong the survival of patients after operation, especially patients with negative margins or lymph nodes. Furthermore, standard regimens of adjuvant have not been identified. This review summarizes the currently available evidence of the effect of adjuvant therapy in the management of EHCC. Ultimately, we concluded that adjuvant therapy may improve survival in high-risk (positive margin or lymph node or advanced stage) patients and adjuvant concurrent chemoradiotherapy followed by chemotherapy may be the optimum selection for them. This needs to be verified by randomized prospective clinical trials.