[Surgical treatment of distal cholangiocarcinoma]

Distal cholangiocarcinoma accounts for about 20% of bile duct cancers, representing the third most common tumor entity among periampullary cancers, along with adenocarcinoma of the pancreas and carcinomas of the papilla of Vater. Precise diagnostics of tumor localization and exact knowledge of tumor-specific growth patterns are crucial for successful surgery. When planning the surgical procedure, the differential diagnosis of central bile duct cancer (Bismuth type I) or middle bile duct carcinoma must be considered. Although benign periampullary bile duct stenosis occurs in only 5% of cases, the presence of immunoglobulin G4-associated cholangitis (IAC) should be excluded in doubtful cases. Basically, in the presence of a distal cholangiocarcinoma, partial pancreatoduodenectomy is indicated analogous to the procedure for ductal adenocarcinoma of the pancreatic head. The 5‑year survival after resection is 20-25% and therefore comparable to adenocarcinoma of the pancreas. Local resection of middle bile duct carcinoma is no longer recommended due to inadequate surgical radicality. To date, perioperative treatment for downsizing does not play a relevant role for surgical treatment of distal cholangiocarcinoma. In the presence of a distal cholangiocarcinoma primary surgery with the aim of a R0 resection is the standard treatment of choice.