Biphenotypic Tumors

Primary liver cancer is broadly recognized as a spectrum marked by hepatocellular carcinoma (HCC) at one end, intrahepatic cholangiocarcinoma (iCCA) at the other end, and biphenotypic or combined hepatocellular-cholangiocarcinoma (cHCC-CCA) in the middle. The WHO criteria exist for the diagnosis and pathological classification of the disease. The cHCC-CCA tumors are uncommon, and a general paucity of data exists regarding the management of patients’ suffering from the disease. Unfortunately, the prognosis for patients suffering from the disease is poor, with a significant rate of recurrence after surgical management of the disease, including treatment by hepatic resection or liver transplantation. For patients with advanced disease, no clear standard exists for palliative systemic therapy. This chapter provides an overview of the epidemiology of cHCC-CCA, the pathologic and radiologic features of the disease involved in making the diagnosis, and the concepts underlying and options for management of the disease.