Treatment strategy for mucin-producing intrahepatic choiangiocarcinoma: Value of percutaneous transhepatic biliary drainage and cholangioscopy

Intrahepatic cholangiocarcinomas that secrete macroscopically excessive muc in into the biliary system are rare, and few of the previously reported cas es have achieved a curative resection. We defined these tumors as "mucin-pr oducing intrahepatic cholangiocarcinomas'' and clarify the optimal preopera tive and surgical management for them. Eleven patients with mucin-producing intrahepatic cholangiocarcinomas underwent surgical resection in our depar tment. The clinical, radiologic, surgical, and pathologic findings were stu died. The clinical presentation of the 11 patients included repeated abdomi nal pain, jaundice, and fever. Conventional cholangiographies, such as perc utaneous transhepatic cholangiography or endoscopic retrograde cholangiogra phy, could not offer precise information about tumor location and extension because of abundant mucin in the biliary system. Using percutaneous transh epatic biliary drainage (PTBD) and percutaneous transhepatic cholangioscopy (PTCS), we were able to drain the mucin and determine precisely the cancer extension into intrahepatic segmental bile ducts. Based on these findings, various types of liver resection,vith or without extrahepatic bile duct re section were planned, and 10 patients obtained curative resection. The cumu lative 5-year survival rate after curative resection was 78%. In patients w ith mucin-producing intrahepatic cholangiocarcinoma, PTBD and PTCS are impo rtant for evaluating the cancer extension. Rational surgery based on accura te preoperative diagnosis improved the prognosis of patients with this dise ase.