Abstract
INTRODUCTION: Hepatocellular carcinoma is the most common form of primary liver cancer. Intrahepatic cholangiocarcinoma and fibrolamellar carcinoma make up most other cases. The vast majority of intrahepatic cholangiocarcinoma's are adenocarcinoma in nature. Few reports have indicated pure squamous cell or mixed squamous glandular histopathology. PRESENTATION OF CASE: We present the case of a 35-year-old female whose preoperative diagnosis indicated primary keratinizing squamous cell carcinoma (SCC) of the liver. However, histological analysis of surgical resections later confirmed intrahepatic cholangiocarcinoma composed of 95 % squamous and 5 % glandular features. DISCUSSION: The change in diagnosis post-operatively is indicative of the pre-operative diagnostic difficulties associated with these newly classified variants. While adenomatous differentiation is the most common form of intrahepatic cholangiocarcinoma, a squamous and mixed histology can be observed. CONCLUSION: Surgeons must be aware of new histological variants of cholangiocarcinoma, potential differentials, and direct further research to improve their poor prognosis.