Abstract
BackgroundNeuroendocrine carcinoma (NEC) originating from the extrahepatic bile duct (EHBD) is very rare but is known for its aggressiveness and poor prognosis. We herein report a case of rapidly progressed NEC in the extrahepatic bile duct.Case presentationAn 84-year-old man was referred to our facility with obstructive jaundice and abdominal pain. Imaging studies revealed an irregular filling defect in the middle bile duct by endoscopic retrograde cholangiopancreatography and an enhanced wall thickening from the middle to distal portion by enhanced computed tomography. The patient was initially diagnosed with extrahepatic cholangiocarcinoma by a bile duct biopsy and underwent pancreatoduodenectomy with lymph node dissection. The pathological findings showed an NEC with an adenosquamous carcinoma component in the extrahepatic bile duct with lymph node metastases. The patient experienced multiple liver metastases 1 month after surgery and died 3 months after surgery. Due to the rapid progression of his disease, his general condition deteriorated, and he was unable to receive any additional treatments, such as chemotherapy.ConclusionAs shown in our case, NEC of the EHBD has an extremely poor prognosis and can sometimes progress rapidly. Multimodality treatment should be considered, even in cases of locoregional disease.
Highlights
Neuroendocrine carcinoma (NEC) originating from the extrahepatic bile duct (EHBD) is very rare but is known for its aggressiveness and poor prognosis
In the WHO 2019 classification, Neuroendocrine neoplasm (NEN) of digestive system are classified into Neuroendocrine tumor (NET) and NECs according to their clinical and molecular differences [7]
The serum levels of carcinoembryonic antigen (CEA) and CA 19-9 were abnormally elevated in some cases, regardless of the presence of adenocarcinoma component, but these are not specific tumor markers for biliary NECs
Summary
We reported a case of resected EHBD NEC with a rapid disease course and poor prognosis. The preoperative diagnosis of primary EHBD NEC is very difficult, and this lesion has an extremely poor prognosis and can progress rapidly after surgery. Multimodality treatment including chemotherapy (neoadjuvant rather than adjuvant), radiotherapy, and surgery should be carefully considered to prolong the survival of patients with EHBD NEC
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