Abstract
BackgroundLarge-cell neuroendocrine carcinoma (LCNEC) of the bile duct is extremely rare and is a high-grade type of neuroendocrine tumor with an aggressive clinical course. Here, we report a case of LCNEC of the extrahepatic bile duct.Case presentationAn 80-year-old man presented with severe jaundice. Endoscopic retrograde cholangiography and enhanced computed tomography revealed complete obstruction of the common bile duct (CBD) by a dense tumor measuring 1.5 cm in diameter. Although there were no malignant cells in the biliary brush cytology, we suspected a cholangiocarcinoma and performed extrahepatic bile duct resection. Histologically, the LCNEC occupied most of the places deeper than the stratum submucosum and an adenocarcinoma component, approximately 15%, was present in the mucosa. There were no transitional areas between the two components. Immunohistochemically, the LCNEC cells were reactive for CD56 and synaptophysin and had a high MIB-1 index (72%). The patient died of multiple liver, lung, and peritoneal metastases 3 months after surgery.ConclusionsLCNEC of the CBD is particularly rare and has a very poor prognosis. Only five cases have been reported in the literature; therefore, there is no established effective therapy, including surgery, for LCNEC of the CBD at present. An accumulation of additional cases and further studies of multimodal treatment are required in the future to improve the prognosis.
Highlights
Large-cell neuroendocrine carcinoma (LCNEC) of the bile duct is extremely rare and is a high-grade type of neuroendocrine tumor with an aggressive clinical course
Five cases have been reported in the literature; there is no established effective therapy, including surgery, for LCNEC of the common bile duct (CBD) at present
An abdominal computed tomography (CT) scan revealed an enhanced mass that was approximately 2.5 cm in size located in the mid common bile duct (CBD) and an enlarged regional node in the hepatoduodenal ligament (Fig. 1a). 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) revealed high accumulation of FDG with a maximum standardized uptake value (SUVmax) of 20.7 by the CBD tumor (Fig. 1b)
Summary
We report a case of LCNEC of the CBD. This disease is extremely rare and has an aggressive malignant potential, including invasiveness and metastasis. There are no effective treatments, including resection. Accumulation of more cases and further studies of multimodal treatment are required to improve the prognosis of patients with this disease. Abbreviations CA19-9: Carbohydrate antigen 19-9; CBD: Common bile duct; CEA: Carcinoembryonic antigen; CT: Computed tomography; ERCP: Endoscopic retrograde cholangiopancreatography; FDG-PET: 18F-fluorodeoxyglucose positron emission tomography; LCNEC: Large-cell neuroendocrine carcinoma; NET: Neuroendocrine tumor; SCNEC: Small-cell neuroendocrine carcinoma; SUVmax: Maximum standardized uptake value
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