What is the role of tumor markers in the diagnosis of cholangiocarcinoma (CCC)?

Updated: Dec 28, 2019
  • Author: Peter E Darwin, MD; Chief Editor: N Joseph Espat, MD, MS, FACS  more...
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A variety of markers have been tested in bile and serum, with limited success. This becomes a significant issue in primary sclerosing cholangitis (PSC), whose clinical features and imaging findings overlap with those of cholangiocarcinoma.

The tumor marker carbohydrate antigen 19-9 (CA 19-9) can be evaluated in pancreatic and bile duct malignancies, as well as in benign cholestasis. A serum CA 19-9 level greater than 100 U/mL (normal < 40 U/mL) has 75% sensitivity and 80% specificity in identifying patients with PSC who have cholangiocarcinoma. [15]

In PSC, an index of carcinoembryonic antigen (CEA) and CA 19-9, has an accuracy of 86% using the following formula: CA 19-9 + (CEA × 40). In a study of patients with PSC, values were over 400 U in 10 of the 15 cases of cholangiocarcinoma but in none of 22 comparable cases with no tumor.  [18]

Bergquist et al reported that in patients with intrahepatic cholangiocarcinoma, an elevated CA 19-9 level is an independent risk factor for mortality. In their study of 2816 patients, those with elevated CA19-9 had more nodal metastases and decreased stage-specific survival. Patients with CA19-9 elevation were less likely to undergo resection, and those who underwent resection had decreased long-term survival. CA19-9 elevation independently predicted increased mortality with an impact similar to that of node positivity, positive-margin resection, and non-receipt of chemotherapy. [19]

Li et al reported that in patients with perihilar cholangiocarcinoma, the preoperative bilirubin level may effectively reflect the severity of disease and provide prognostic information. In patients with a low bilirubin (≤12 mg/dL), 1-, 3-, and 5-year overall survival rates after resection were 75.9%, 36.5%, 21.7%; in those with bilirubin > 12 mg/dL, corresponding overall survival rates were 53.6%, 13.9%, and 0%. In addition, rates of early tumor recurrence were significantly higher in patients with high preoperative bilirubin levels than in those with lower levels. [20]

Cholangiocarcinoma does not produce alpha-fetoprotein.

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