title: Cholangiocarcinoma and high-grade dysplasia in young patients with primary sclerosing cholangitis.
abstract: BACKGROUND
  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.
  
  
  OBJECTIVE
  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.
authors.affiliation: Gastroenterology and Hepatology, Huntsman Cancer Center, University of Utah School of Medicine, 30 N 1900E 4R118, Salt Lake City, UT, 84312, USA.
authors.forename: Roy
authors.initials: R
authors.lastname: Liu
authors.forename: Kristen
authors.initials: K
authors.lastname: Cox
authors.forename: Stephen L
authors.initials: SL
authors.lastname: Guthery
authors.forename: Linda
authors.initials: L
authors.lastname: Book
authors.forename: Benjamin
authors.initials: B
authors.lastname: Witt
authors.forename: Barbara
authors.initials: B
authors.lastname: Chadwick
authors.forename: Douglas G
authors.initials: DG
authors.lastname: Adler
mesh_terms: D000293:Adolescent; D001650:Bile Duct Neoplasms / Q000150:complications / Q000556:secondary* / Q000628:therapy; D001653:Bile Ducts, Intrahepatic; D018281:Cholangiocarcinoma / Q000150:complications / Q000473:pathology* / Q000628:therapy; D002760:Cholangiopancreatography, Endoscopic Retrograde; D049448:Cholangiopancreatography, Magnetic Resonance; D015209:Cholangitis, Sclerosing / Q000150:complications / Q000175:diagnosis*; D003093:Colitis, Ulcerative / Q000150:complications; D003424:Crohn Disease / Q000150:complications; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D012189:Retrospective Studies
references.citation: Hum Pathol. 2013 Oct;44(10):2173-9
references.pmid: 23845469
references.citation: Int J STD AIDS. 2008 Oct;19(10):717-8
references.pmid: 18824629
references.citation: J Immunol. 1997 Jan 15;158(2):977-83
references.pmid: 8993019
references.citation: Cancer Cytopathol. 2013 Dec;121(12):708-17
references.pmid: 23839915
references.citation: World J Gastrointest Oncol. 2013 Jul 15;5(7):132-8
references.pmid: 23919107
references.citation: Am J Dis Child. 1977 Apr;131(4):442-4
references.pmid: 192070
references.citation: Am J Gastroenterol. 2007 Jan;102(1):107-14
references.pmid: 17037993
references.citation: Gastroenterology. 2006 Oct;131(4):1064-72
references.pmid: 17030177
references.citation: Hepatology. 2000 Jan;31(1):247-8
references.pmid: 10613754
references.citation: Clin Gastroenterol Hepatol. 2007 Oct;5(10):1221-8
references.pmid: 17689296
references.citation: Ann Surg. 1991 Jan;213(1):21-5
references.pmid: 1845927
references.citation: Am J Gastroenterol. 2007 Aug;102(8):1677-82
references.pmid: 17433022
references.citation: Gut. 1996 Apr;38(4):610-5
references.pmid: 8707097
references.citation: Gastroenterology. 2005 Mar;128(3):620-6
references.pmid: 15765398
references.citation: J Pediatr Gastroenterol Nutr. 2011 May;52(5):617-20
references.pmid: 21502832
references.citation: Hepatogastroenterology. 1990 Aug;37(4):395-7
references.pmid: 2170255
references.citation: Ann Oncol. 1999;10 Suppl 4:94-8
references.pmid: 10436795
references.citation: J Pediatr Surg. 2006 Jan;41(1):e15-9
references.pmid: 16410082
references.citation: Am J Gastroenterol. 2006 Feb;101(2):284-91
references.pmid: 16454832
references.citation: Am J Gastroenterol. 2011 Nov;106(11):2023-8
references.pmid: 21844920
references.citation: Hepatology. 1998 Feb;27(2):311-6
references.pmid: 9462625
references.citation: J Pediatr Surg. 2009 Nov;44(11):2202-5
references.pmid: 19944233