Patients diagnosed with cholangiocarcinoma (CCA) between the ages of 18 and 49 years may represent a demographically and clinically distinct group from patients with more a typical onset of disease after age 50, according to the largest analysis of patients with young-onset CCA to date.

Information is limited about patients who are diagnosed with CCA before 50 years of age. In the present study published in Cancer Medicine, a team of researchers led by Syed Kazmi, MD, from the Division of Hematology and Oncology at UT Southwestern Medical Center in Dallas, Texas compared the clinical characteristics and outcomes of patients with CCA diagnosed before and after age 50.

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The team used data from the National Cancer Database and identified 2520 patients diagnosed with the disease between 18 and 49 years of age and 23,826 patients diagnosed after age 50. 

They found that patients with young-onset CCA were less likely to be White than those with typical-onset disease. Patients with young-onset disease also had a lower overall comorbidity burden. 

Moreover, the proportions of intrahepatic CCA and stage 4 disease were greater among patients with young-onset disease than those with a typical onset. 

In terms of treatment, patients with young-onset disease were more likely to receive definitive surgery, radiation, and chemotherapy than patients with typical-onset disease. 

Finally, the researchers reported that following adjusted analyses, patients with young-onset CCA had a 15% lower risk of death than those with typical-onset CCA.

“Further research on the nature of cholangiocarcinoma in the young, including symptom burden, molecular profile, and efficacy and tolerability of specific treatment regimens, is warranted,” the researchers wrote.

CCA is a heterogeneous group of rare malignant tumors originating from biliary tree cells. It comprises 15% of all primary liver tumors and is the second most common primary malignancy of the liver after hepatocellular carcinoma.


Reddy S, Goksu SY, Sanford NN, et al. Characteristics and clinical outcomes in young-onset cholangiocarcinoma. Cancer Med. Published online May 22, 2023. doi:10.1002/cam4.6063