Clear cell papillary cholangiocarcinoma: a case report with review of literature

Clear cell papillary cholangiocarcinoma is a rare variant of intrahepatic cholangiocarcinoma with only nine reported cases in the literature. This tumor needs to be differentiated from clear cell variant of hepatocellular carcinoma and other metastatic clear cell carcinomas. This tumor is known to have better prognosis compared to conventional intrahepatic cholangiocarcinoma. Hence, it is important to correctly diagnose this entity and differentiate it from other histologic mimics. We describe clinical, histopathological, and immunohistochemical findings of one such rare case in a 66-year-old gentleman who was incidentally detected to have an intrahepatic tumor at routine ultrasound examination.A review of all the similar cases reported so far in the literature is also provided.