Sarcomatoid Intrahepatic Cholangiocarcinoma: A Rare and Aggressive Primary Liver Cancer

Sarcomatoid intrahepatic cholangiocarcinoma (S-iCCA) is a rare variant of primary liver cancer with a poor prognosis due to local aggressive expansion and frequent metastases. The pathogenesis remains unclear, but theories suggest epithelial-mesenchymal transition, biphasic differentiation of pluripotent stem cells, or sarcomatoid re-differentiation of immature multipotent carcinoma cells. Chronic hepatitis B and C, cirrhosis, and age above 40 are plausible contributors. Diagnosis of S-iCCA requires immunohistochemical evidence of both mesenchymal and epithelial molecular expression. Early detection and total resection are the current mainstay approach. We report a case of metastatic S-iCCA in a 53-year-old male with alcohol use disorder who underwent en bloc right hepatic lobectomy, right adrenalectomy, and cholecystectomy.