OBJECTIVES: Cholangiocarcinoma (CCA) is a biliary malignancy found primarily in adults. The incidence of CCA in children is unknown. The aim of this study was to describe characteristics of CCA in children and adolescents.
METHODS: Using the Surveillance, Epidemiology, and End Results Program (SEER 18) database, we identified incident cases of CCA diagnosed at <20 years of age during the period of 1973-2013. Additionally, we reviewed published case reports describing pediatric patients with CCA. We calculated descriptive statistics for CCA cases identified in SEER and in case reports. Kaplan-Meier analysis was performed to determine median and three-year overall survival (OS) rates.
RESULTS: We identified 15 children and adolescents diagnosed with CCA from SEER 18 with an incidence rate of 0.0036/100,000. Two-thirds of cases were male, and the majority Caucasian (n=10). The median age at diagnosis was 17 years (range: 11-19 years). Nine tumors were intrahepatic, three extrahepatic, and three unspecified. One-third had distal metastases at diagnosis. Eight patients underwent surgical resection including liver transplant in two. Six patients were alive at time of follow-up. Patients without surgical treatment did not survive. Three-year OS was 50%. Twenty-two children with CCA were found in the literature with a median age at diagnosis of 15 years (range: 3-18 years). Half were male, and 90% had an underlying gastrointestinal comorbidity. Three-year OS was 35.3%.
CONCLUSIONS: CCA in children and adolescents is rare with poor survival. A high proportion of cases had a history of biliary disease. Surgical resection is necessary for cure.
