Mucin-producing cholangiocarcinoma: clinical experience of 24 cases in 16 years.
OBJECTIVE
Mucin-producing cholangiocarcinoma (MPCC) is an uncommon biliary tract malignancy. This retrospective study was designed to investigate the clinical presentations, associated diseases, the value of tumor markers, and treatment and prognosis of MPCC.


METHODS
The study included 24 MPCC cases (7 M, 17 F) from 132 histopathology-proven cholangiocarcinoma patients seen between 1988 and 2003. MPCC and non-mucin-producing cholangiocarcinoma (non-MPCC) were compared.


RESULTS
Common clinical symptoms and signs included epigastric or right upper quadrant pain, fever and chills, jaundice, and anemia. Seventeen patients had mucobilia in surgical, cholangiographic, or CT findings. Biliary stones were found in 71% of MPCC patients. In detecting MPCC, a serum CA-19-9 level > 120 U/ml had 73% sensitivity, 41% specificity, 25% positive predictive value, 85% negative predictive value, and 48% accuracy in the 132 cholangiocarcinoma patients. The 1-, 2-, 3-, 4-, and 5-year survival rates were 44.3%, 33.8%, 28.1%, 21.1%, and 21.1%, respectively. Univariate analysis showed that tumor type (MPCC) was the only significant factor predicting survival when compared with non-MPCC patients in 5-year cumulative survival rates (21.1% versus 5.4%). MPCC patients receiving surgical resection had a prolonged median survival time (13 months versus 6 months) than those without resection.


CONCLUSIONS
MPCC clinical presentations were similar to non-MPCC in Taiwan except for more jaundice, fever and chills. The major factor predisposing to MPCC was biliary stones. Using serum CA19-9 levels greater than 120 U/ml was a diagnostic aid to detecting MPCC patients. MPCC patients had a better prognosis than non-MPCC patients. The recommended treatment for MPCC is surgical resection.
Kuo
Chung-Mou
CM
Division of Gastroenterology, Department of Internal Medicine, Chang Gung Memorial Hospital, Kaohsiung Medical Center, Taiwan. kuo25612@ms2.hinet.net
Changchien
Chi-Sin
CS
Wu
Keng-Liang
KL
Chuah
Seng-Kee
SK
Chiu
King-Wah
KW
Chiu
Yi-Chun
YC
Chou
Yeh-Pin
YP
Kuo
Chung-Huang
CH
D000328:Adult; D000368:Aged; D001650:Bile Duct Neoplasms / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D001653:Bile Ducts, Intrahepatic; D001662:Biliary Tract Surgical Procedures; D014408:Biomarkers, Tumor / Q000097:blood; D018395:CA-19-9 Antigen / Q000097:blood*; D018281:Cholangiocarcinoma / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D002769:Cholelithiasis / Q000150:complications; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009077:Mucins / Q000502:physiology*; D011237:Predictive Value of Tests; D011379:Prognosis; D012189:Retrospective Studies
D014408:Biomarkers, Tumor; D018395:CA-19-9 Antigen; D009077:Mucins