We report on a patient with biliary cystadenocarcinoma and review 112 previ
ously published cases of this rare cystic hepatic neoplasm. This tumour mai
nly occurs in women at a ratio of 62% (female) to 38% (male), and at an ave
rage age of 56.2 years (range 18-88 years). The origin of these neoplasms i
s intrahepatic in 97% of cases and extrahepatic in the remaining 3%. The cl
inical symptoms are nonspecific and are not distinctive from benign cystic
liver lesions unless invasive growth of the tumour occurs or distant metast
ases are present. Sonography and computed tomography (CT), as well as magne
tic resonance imaging (MRI) demonstrate the multilocular nature of the tumo
ur with septal or mural nodules. Discrete soft tissue masses, thick and coa
rse calcifications and varying density on CT or intensity on MRI within the
loculi are additional non-specific imaging findings. The best therapeutic
result with a 5-year survival rate of 100% and a recurrence rate of only 13
% was achieved by complete excision (n=16). Surgical removal of the tumour
by complete excision is, therefore, the treatment of choice for biliary cys
tadenocarcinomas. (C) 1998 Elsevier Science Ltd. All rights reserved.