Mucin-producing cholangiocarcinoma: clinical experience of 24 cases in 16 years

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.

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.

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.

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.