The characteristic features of surgically curable mucin-producing extrahepa
tic 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, di
agnostic procedures and operative methods for patients with MPEBC were inve
stigated. Tumors in the bile duct were identified by cholangioscopy combine
d with cholangiography after removal of mucin balls. Tumors were located cl
ose to the hepatic confluence in these patients. Two patients underwent hep
atic lobectomy together with caudate lobectomy while the other underwent re
section 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 dise
ase. Accurate localization in the biliary tree is essential and can only be
obtained after, i) removal of mucin balls, and ii) extensive diagnostic wo
rk-up including cholangiography, cholangioscopy and intraoperative patholog
ical examination.