| [ Summary ] |
We defined cholangiocarcinoma secreting macroscopically excessive amounts of mucin into the biliary system as "mucin-producing cholangiocarcinoma", and presented the clinical features and the outcome of 11 surgically treated cases. The clinical presentations of the 11 patients included repeated abdominal pain, jaundice, and fever. Conventional cholangiographies such as percutaneous transhepatic cholangiography or endoscopic retrograde cholangiography could not offer precise information about tumor location and extension because of abundant mucin in the biliary system. After draining mucin through percutaneous transhepatic biliary drainage (PTBD), we achieved clear cholangiograms and determined the cancer extension precisely. Percutaneous transhepatic cholangioscopy (PTCS) was indispensable for investigating the superficial spread of cancer. Based on these findings, all tumors were resected surgically, and 10 patients obtained curative resection. Macroscopically, papillary tumors developed in diffusely dilated intrahepatic bile ducts in 4 cases, whereas, Iarge cystic lesions with papillary projections developed in the liver in other 7 cases. The superfical spread of the cancer contiguous to the primary tumor was observed in 5 of 11 cases. Postoperatively, 7 patients survived for at least 5 years and the median survival rate was 10.8 years. Rational surgery procedures based on accurate preoperative diagnosis using PTBD and PTCS improved the prognosis of patients with this disease. |