A 65-year-old man was hospitalized due to an abdominal tumor. Several
imaging studies showed multilocular tumors in the right hepatic lobe a
nd in the pancreatic head, 4 and 7 cm in size, respectively. The hepat
ic tumor rapidly grew to 9 cm in 4 weeks, associated with a rapid incr
ease in the serum carcinoembryonic antigen level from 125 ng/ml to 1,0
00 ng/ml. The pathologic diagnosis of the resected liver tumor was muc
inous cholangiocarcinoma. This hepatic tumor produced a large amount o
f mucin, but did not secrete mucin into the bile ducts. Therefore, the
re was no obstructive jaundice. The incidentally accompanied pancreati
c tumor was a typical serous cystadenoma. The present case suggested t
hat mucinous cholangiocarcinoma could present rapid growth of the tumo
r. The clinicopathological features of mucinous cholangiocarcinoma are
demonstrated for the first time, and the differences between mucinous
cholangiocarcinoma and mucin-producing papillary adenocarcinoma of du
ct-spreading type are discussed.