Background: PSC is a progressive cholestatic liver disease, without effective medical treatment, which is highly associated with CCA. In end stage disease, survival can be prolonged with liver transplantation. Diagnosing CCA in PSC is difficult and has implications in the timing and outcome of transplantation. We report the largest series of BC and serum tumor markers for the diagnosis of CCA in PSC. Patients and Methods: We identified 333 PSC patients in whom a definitive diagnosis of CCA (explant, autopsy, biopsy, long term follow-up) could be obtained or excluded. 318 BC specimens were obtained at ERCP (263) or PTC (55) in 151 patients, and serum CEA and CA19-9 were available in 114 and 45 patients respectively. 42 patients had both serum markers and BC. Cytology was considered positive only when the pathologist reported the presence of cancer. We defined abnormal values for CEA as > 10 ng/ml and CA19-9 as > 200 U/ml. Results: The incidence of CCA was 13.2% (44/333). CCA was diagnosed at a mean of 3.2 years following the diagnosis of PSC. CCA was not increased in PSC associated with IBD versus idiopathic PSC. Sensitivity (SENS), specificity (SPEC), positive predictive value (PPV), negative predictive value (NPV) and diagnostic accuracy (ACC) of each test and all combinations of tests were calculated. The most accurate combination was BC or CA19-9. SENS (%) SPEC (%) PPV (%) NPV (%) ACC (%) BC (n=151) 46 100 100 89 90 CA19-9 (n = 45) 77 97 87 94 93 CEA(n=114) 50 95 73 88 87 BC or CA19-9 (n = 42) 86 97 86 97 95 Only 4/7 patients with cytology highly suspicious for malignancy or severe dysplasia had CCA, and inclusion of these interpretations as positive for CCA did not improve diagnostic accuracy. Of the 13 CCA diagnosed by BC, 9 were detected at the 1st brush, 2 at the 2nd and 2 at the 3rd Conclusion: The combination of BC or CA19-9 can detect CCA with reasonable accuracy. These data are useful in developing strategies to monitor patients with PSC for the development of CCA.
|Original language||English (US)|
|State||Published - Dec 1 1998|
ASJC Scopus subject areas
- Radiology Nuclear Medicine and imaging