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Semin Liver Dis 2012; 32(04): 360-366
DOI: 10.1055/s-0032-1329904
Diagnostic Problems in Hepatology
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Cholangiolocellular Carcinoma in a Pediatric Patient with Small Duct Sclerosing Cholangitis: A Case Report

Joanne Lai
1   Division of Hepatology, Department of Pediatrics and RMTI, Department of Surgery, The Mount Sinai Medical Center, New York, New York
,
Bashir Taouli
2   Department of Radiology, The Mount Sinai Medical Center, New York, New York
,
Kishore R. Iyer
1   Division of Hepatology, Department of Pediatrics and RMTI, Department of Surgery, The Mount Sinai Medical Center, New York, New York
,
Ronen Arnon
1   Division of Hepatology, Department of Pediatrics and RMTI, Department of Surgery, The Mount Sinai Medical Center, New York, New York
,
Swan N. Thung
3   The Lillian and Henry M Stratton – Hans Popper Department of Pathology, The Mount Sinai Medical Center, New York, New York
,
Margret S. Magid
3   The Lillian and Henry M Stratton – Hans Popper Department of Pathology, The Mount Sinai Medical Center, New York, New York
,
Nanda Kerkar
1   Division of Hepatology, Department of Pediatrics and RMTI, Department of Surgery, The Mount Sinai Medical Center, New York, New York
4   Department of Gastroenterology, Children's Hospital of Los Angeles and Keck School of Medicine, University of southern California, Los Angeles, California
› Author Affiliations
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Publication History

Publication Date:
08 February 2013 (online)

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Abstract

Although guidelines exist for routine screening for malignancy in adults with primary sclerosing cholangitis, no imaging guidelines exist for the pediatric population. Cholangiolocellular carcinoma is a rare malignant liver tumor that has been found in adults with chronic liver disease. We present a case of cholangiolocarcinoma found in an adolescent boy with small duct sclerosing cholangitis. The diagnosis of small duct sclerosing cholangitis was made at the age of 6 at which time he also had advanced fibrosis histologically, but no evidence of decompensation either clinically or biochemically. Several years after this diagnosis, a small liver lesion was found incidentally on computed tomography scan following a motorcycle accident. This lesion was shown to be stable by magnetic resonance imaging over the course of 2 years. At 15 years of age, magnetic resonance imaging findings changed with features suggestive of malignancy. This led to resection of the lesion. Pathologic examination confirmed the presence of cholangiolocarcinoma, a tumor found primarily in adults with a history of viral hepatitis. To our knowledge, this is the first such report in a pediatric patient.

Keywords

cholangiolocellular carcinoma - small duct sclerosing cholangitis - pediatric
 

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