LAUSR

In young patients, 81%, 38%, 87%, 7%, 40%, and 67% of tumors were positive for CK7, CK20, CK19, NCAM, N-Cadherin, S100P, compared to 95%, 32%, 95%, 0%, 52%, and 38% in the control group, respectively. Diffuse strong expression of p53 was observed in 33% of tumors of the young (vs 52% in control group). All tumors had intact expression of mismatch repair proteins. Mean follow-up time was 2.8 years (range 0.25–11 years). Fourteen (70%) patients died of disease, and the five-year survival rate was 25%. Conclusion: Cholangiocarcinoma of the young is highly aggressive, most commonly presenting at advanced stage and with high histologic grade, and is hence associated with poor survival. It is more commonly intrahepatic than cholangiocarcinoma in older adults, but is morphologically and immunohistochemically similar to it, except for increased expression of S100P and decreased expression of p53.