Intrahepatic cholangiocarcinoma is a cancer of the bile duct (a cholangiocarcinoma) that arises inside the liver. It accounts for 10% of primary liver cancer, though it is responsible for a much larger proportion in parts of China and southeast Asia where liver flukes (Clonorchis sinensis and Opisthorchis viverrini) are common.
Usually, older adults are more affected, with no gender differences. In North America, the disease is most commonly associated with ulcerative colitis, primary sclerosing cholangitis, and congenital anomalies of the biliary tree. Other factors include hepatolithiasis, liver flukes, and thorotrast - a long-ago used contrast agent. Unlike several other liver carcinomas, this one is not associated with cirrhosis.
The most probable sequence of development is hyperplasia of the bile duct epithelium followed by dysplasia, carcinoma in situ, and then invasive carcinoma. On gross examination, the carcinoma is hard and white and usually an adenocarcinoma. Microscopically, it consists of malignant glands lined by cells resembling biliary epithelium.
Diagnosis of these tumors is based on histology. Usually, patients present very late with right upper quadrant pain (47%), weight loss, obstructive jaundice (90%) and weakness. The tumours are usually unresectable, therapy is usually of little value, and the prognosis is poor, with a five year survival of less than 10%.