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

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Abstract

Introduction

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.

Aim

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

Methods

Retrospective study

Results

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.

Conclusions

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

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Authors and Affiliations

  1. Gastroenterology and Hepatology, Huntsman Cancer Center, University of Utah School of Medicine, 30 N 1900E 4R118, Salt Lake City, UT, 84312, USA

    Roy Liu, Kristen Cox & Douglas G. Adler

  2. Division of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, University of Utah School of Medicine, Salt Lake City, UT, USA

    Stephen L. Guthery & Linda Book

  3. Department of Pathology, University of Utah School of Medicine, Salt Lake City, UT, USA

    Benjamin Witt & Barbara Chadwick

Authors
  1. Roy Liu
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  2. Kristen Cox
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  3. Stephen L. Guthery
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  4. Linda Book
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  5. Benjamin Witt
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  6. Barbara Chadwick
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  7. Douglas G. Adler
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Correspondence to Douglas G. Adler.

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Liu, R., Cox, K., Guthery, S.L. et al. Cholangiocarcinoma and High-Grade Dysplasia in Young Patients with Primary Sclerosing Cholangitis. Dig Dis Sci 59, 2320–2324 (2014). https://doi.org/10.1007/s10620-014-3152-0

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  • DOI: https://doi.org/10.1007/s10620-014-3152-0

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