Primary hepatic neuroendocrine carcinoma with a cholangiocellular carcinoma component in one nodule

Primary neuroendocrine carcinomas (NECs) in the liver are very rare; however, several reports have described cases of a primary hepatic NEC combined with a hepatocellular carcinoma (HCC). We present the first report of a primary hepatic NEC with a cholangiocellular carcinoma (CCC) component in one nodule in a patient with a metachronous liver HCC. A 73-year old man who had received partial hepatectomy surgery because of a primary HCC and a primary CCC two years prior was diagnosed with a primary hepatic NEC after surgical treatment. Histological analysis of the resected tumor revealed that the tumor consisted of a predominant NEC area with a partial CCC component in one nodule and that the NEC cells were negative for markers of pancreatic NEC. Neoplastic cells in both the NEC and CCC component focally expressed CD44, a representative marker for cancer-initiating cells, and the CD44-positive cells in the NEC component were seen in the vicinity of those in the CCC component of one nodule. This case report provides suggestive information for the origin of primary hepatic NECs.