We report herein an unusual case of a composite glandular-neuroendocri
ne carcinoma of the hilar bile duct. A 71-year-old Japanese woman was
admitted to our hospital suffering from general fatigue, progressive j
aundice, and a high fever. Computed tomography and angiography finding
s revealed a solid hypervascular mass in the hepatic hilus, Thus, a su
bsegmentectomy of the liver (S-4, S-5) and bile duct resection with ly
mphnode dissection were performed. A tumor measuring 6.0 x 3.0 cm was
found to be located in the bile duct of the hepatic hilus. Histologica
lly, the tumor was composed of well-differentiated adenocarcinoma and
small cell neuroendocrine carcinoma cells, with a histological transit
ion between the two components, Grimelius' method revealed the presenc
e of diffuse positive tumor cells in neuroendocrine carcinoma, The neu
roendocrine tumor cells were also diffusely immunoreactive to chromogr
anin A, To the best of our knowledge, only 22 previous cases of compos
ite glandular-neuroendocrine carcinoma in the biliary tract have been
reported; however, this is the first case report of a clearly composit
e tumor of the hilar bile duct.