Digestive Diseases and Sciences

, Volume 59, Issue 9, pp 2320–2324 | Cite as

Cholangiocarcinoma and High-Grade Dysplasia in Young Patients with Primary Sclerosing Cholangitis

  • Roy Liu
  • Kristen Cox
  • Stephen L. Guthery
  • Linda Book
  • Benjamin Witt
  • Barbara Chadwick
  • Douglas G. Adler
Original Article



Cholangiocarcinoma (CCA) is very often an adulthood disease with primary sclerosing cholangitis (PSC) as one of the risk factors. It is rarely seen in the pediatric population, and when it is diagnosed before adulthood, it can be associated with PSC as well as HIV infection, biliary atresia, radiation therapy, and choledochal cyst. Although there have been some case reports of pediatric CCA, cases of childhood CCA associated with PSC are still relatively rare.


To describe the clinical and pathologic features of CCA in pediatric patients with previously diagnosed PSC.


Retrospective study


Four patients with PSC (age range 15–18, mean 17 years) were included in this study. All patients underwent ERCP for diagnosis. Tissue samples obtained included routine cytology and FISH. ERCP was used to target sites for tissue acquisition in all patients. 3/4 of patients have inflammatory bowel disease (two Crohn’s disease and one ulcerative colitis). Alkaline phosphatase was elevated in 3/4 patients, aspartate aminotransferase/alanine aminotransferase were elevated in 2/4 patients, and total bilirubin/direct bilirubin were elevated in 2/4 patients. 4/4 patients had positive FISH studies, and 3/4 patients had brush cytology concerning for CCA. 2/4 patients received chemotherapy, one patient underwent orthotopic liver transplant, and one patient underwent Whipple procedure. Two patients died soon after being diagnosed.


Young patients with PSC can develop CCA. This finding has implications for both screening and surveillance for cancer in pediatric patients with PSC.


ERCP Cholangiocarcinoma Primary sclerosing cholangitis 


Conflict of interest



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Copyright information

© Springer Science+Business Media New York 2014

Authors and Affiliations

  • Roy Liu
    • 1
  • Kristen Cox
    • 1
  • Stephen L. Guthery
    • 2
  • Linda Book
    • 2
  • Benjamin Witt
    • 3
  • Barbara Chadwick
    • 3
  • Douglas G. Adler
    • 1
  1. 1.Gastroenterology and Hepatology, Huntsman Cancer CenterUniversity of Utah School of MedicineSalt Lake CityUSA
  2. 2.Division of Pediatric Gastroenterology, Hepatology and Nutrition, Department of PediatricsUniversity of Utah School of MedicineSalt Lake CityUSA
  3. 3.Department of PathologyUniversity of Utah School of MedicineSalt Lake CityUSA

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