Cholangiolocellular carcinoma in a pediatric patient with small duct sclerosing cholangitis: a case report

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.