Primary liver cancer: intrahepatic cholangiocarcinoma emerges from the shadows

Excerpt

Intrahepatic cholangiocarcinoma (ICC) is an uncommon malignant neoplasm that arises from intrahepatic bile duct epithelium. ICC accounts for approximately 10% of all primary liver cancers worldwide and is the second most common primary hepatic malignancy after hepatocellular carcinoma (HCC) [1]. In part, due to the relative rarity of the disease, no distinction was made in the sixth edition of the American Joint Committee on Cancer (AJCC)/International Union Against Cancer (UICC) staging manual between ICC and HCC [2]. The appropriateness of this single staging system was controversial, due to the inherent differences in the etiology and biology of the two diseases. Several previous staging systems have been proposed based on data from Japan, where the experience with ICC has been more pronounced. The applicability of these systems to ICC in Western countries, however, was unclear. Recently, the staging of ICC has been revised and improved in the seventh edition of the AJCC/UICC Cancer Staging Manual [3]. The changes address the need for an empiric data-derived staging system for ICC distinct from HCC. The new ICC staging system places an emphasis on multiplicity of tumors, presence of vascular invasion, and lymph node metastasis. …