Mucin ball-producing extrahepatic bile duct carcinoma.

The characteristic features of surgically curable mucin-producing extrahepatic bile duct carcinoma (MPEBC) have not previously been elucidated. Three (6.5%) of 46 patients who underwent surgery in our department for bile duct carcinoma between 1986 and 1997 had MPEBC. Clinicopathological features, diagnostic procedures and operative methods for patients with MPEBC were investigated. Tumors in the bile duct were identified by cholangioscopy combined with cholangiography after removal of mucin balls. Tumors were located close to the hepatic confluence in these patients. Two patients underwent hepatic lobectomy together with caudate lobectomy while the other underwent resection of the hepatic confluence. Absence of residual tumors was confirmed histologically in these patients. All three patients remain alive without evidence of recurrence, 22-54 months after surgery. MPEBC is a curable disease. Accurate localization in the biliary tree is essential and can only be obtained after, i) removal of mucin balls, and ii) extensive diagnostic work-up including cholangiography, cholangioscopy and intraoperative pathological examination.