<p><b>OBJECTIVE</b>To summarize the experience in
diagnosis, prevention and
treatment of
carcinoma arising from
congenital biliary duct
cyst.</p><p><b>
METHODS</b>The clinical and pathological data of 185
patients with
congenital biliary duct
cyst admitted to
Chinese PLA
General Hospital were analyzed retrospectively.</p><p><b>RESULTS</b>Among 185
patients, twenty-seven cases had
carcinomas arising from
congenital biliary duct
cyst, and the frequency of malignant transformation was 14.6%, which closely related to the age (P < 0.001). The
incidences of
malignancy for different
age groups were 0 for 0-9
age group, 5.1% for 0-19, 9.1% for 20-29, 16.2% for 30-39, 26.7% for 40-49, 33.3% for 50-59, and 50% for over 60, respectively. Six
patients had the
history of
cyst-
enterostomy.
Abdominal pain,
fever,
jaundice and
weight loss were the main clinical manifestations. Abdominal
ultrasonography, CT, MRI or
magnetic resonance cholangiopancreatography, MRCP and endoscopic retrograde choledochopancreatography (
ERCP) were the main diagnostic
methods. For twenty
patients (74.1%), a definite
diagnosis was made preoperatively, but it's hard to make an
early diagnosis. Nine
patients (33.3%) underwent curative resection.</p><p><b>CONCLUSIONS</b>
Congenital biliary duct
cyst is a premalignant lesion, and the
incidence of
carcinogenesis increases remarkably with age. The most effective
method for prevention of
carcinogenesis in
choledochal cyst is complete excision of
choledochal cyst during childhood, and the
prognosis is poor for
patients with biliary
malignancy.</p>