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

Chung Mou Kuo, Chi Sin Changchien, Keng Liang Wu, Seng Kee Chuah, King Wah Chiu, Yi Chun Chiu, Yeh Pin Chou, Chung Huang Kuo*

*Corresponding author for this work

Research output: Contribution to journalJournal Article peer-review

12 Scopus citations

Abstract

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. Material and 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.

Original languageEnglish
Pages (from-to)455-459
Number of pages5
JournalScandinavian Journal of Gastroenterology
Volume40
Issue number4
DOIs
StatePublished - 04 2005
Externally publishedYes

Keywords

  • CA19-9
  • Cholangiography
  • Mucin-producing cholangiocarcinoma
  • Mucobilia

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