Distal cholangiocarcinoma.

Distal cholangiocarcinoma is an uncommon malignancy and early diagnosis remains a challenge. More accurate diagnostic modalities for early-stage diagnosis are needed. Advances in medical therapy and neoadjuvant treatment may aid surgery and further improve postoperative outcomes. Margin-negative resection in conjunction with thorough nodal dissection is the strongest prognostic factor. Surgical resection coupled with adjuvant therapy provides the most favorable outcome. Future efforts should be aimed at reducing surgical complications and improving medical therapy, leading to overall improvement in perioperative and long-term outcomes for patients with this disorder.