Published online Jul 28, 2013. doi: 10.3748/wjg.v19.i28.4616
Revised: April 10, 2013
Accepted: April 18, 2013
Published online: July 28, 2013
Neuroendocrine carcinoma (NEC) of the extrahepatic bile duct is rare, and only 22 cases have been reported. Only two of these were large-cell NEC (LCNEC); the vast majority were small-cell NEC. Here, we report a third case of LCNEC of the extrahepatic bile duct. A 76-year-old male presented to a local hospital with painless jaundice. Imaging studies revealed a tumor at the hepatic hilum. The patient underwent right hepatic lobectomy, bile duct resection, and cholecystectomy. The resection specimen showed a 5.0-cm invasive neoplasm involving the hilar bile ducts and surrounding soft tissue. Histologically, the tumor consisted of nests of medium to large cells with little intervening stroma. The tumor invaded a large portal vein branch. All four excised lymph nodes were positive for metastasis, and metastatic deposits were also present in the gallbladder wall. The tumor was diffusely positive for synaptophysin and focally positive for chromogranin A. Approximately 70%-80% of the tumor cells were positive for Ki-67, indicating strong proliferative activity. A diagnosis of LCNEC was made. A few bile ducts within and adjacent to the invasive tumor showed dysplasia of the intestinal phenotype and were focally positive for synaptophysin and chromogranin A, suggesting that the dysplastic intestinal-type epithelium played a precursor role in this case. A postoperative computer tomography scan revealed rapid enlargement of the abdominal and retroperitoneal lymph nodes. The patient died 21 d after the operation. NEC of the bile duct is an aggressive neoplasm, and its biological characteristics remain to be better defined.
Core tip: The authors report a case of large-cell neu-roendocrine carcinoma (LCNEC) of the hilar bile duct. Concurrent dysplasia with intestinal and neuroendocrine differentiation was suggested to be a precursor in this case. Neuroendocrine carcinoma (NEC) of the bile duct occurs more frequently in men (male:female ratio 1.9:1). The mid-portion of the common bile duct appears to be the commonest site of involvement. All three reported cases of LCNEC died within 12 mo and the prognosis of NEC of the bile duct appears to be equally poor in both small-cell NEC and LCNEC. Multimodal treatment may improve outcome in this highly aggressive cancer.