Management of combined hepatocellular carcinoma-cholangiocarcinoma

To review the recent consensus on the nomenclature, clinical features, diagnosis, and treatment of combined hepatocellular carcinoma-cholangiocarcinoma (cHCC-CCA).

cHCC-CCA is a primary liver carcinoma with varying degrees of hepatocytic and cholangiocytic cytology and architecture within the same tumor. The diagnosis of cHCC-CCA can only be established based on histologic examination. Surgical resection should be considered in patients with resectable tumors who do not have underlying liver disease or clinically significant portal hypertension. While treatment by liver transplantation (LT) is controversial due to the high risk of post LT recurrence, LT should remain as a potentially curative option in a highly selected group of patients. Little data exist for the outcome of other treatments.

High quality multicenter prospective studies should be conducted to better understand this rare, but increasingly recognized tumor.