Rapidly progressed neuroendocrine carcinoma in the extrahepatic bile duct: a case report and review of the literature

Abstract

Background: Neuroendocrine 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 presentation: An 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. Conclusion: As 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.

Small-cell Neuroendocrine Carcinoma of the Extrahepatic Bile Duct: A Rare Case Report

“…[6][7][8] NENs have a range of clinical outcomes, including indolent and aggressive features depending on the differentiation and proliferation grading. [6][7][8] NENs are classified as well-differentiated neuroendocrine tumors (NETs), poorly differentiated small-cell and large-cell NECs, and mixed neuroendocrine-non-neuro- In the cases reported in the literature, 9,10 the median age of patients with NEC of the extrahepatic bile duct was 70.0 years old. The sex predisposition was predominantly male.…”

“…The median overall survival was only 12 months (95% confidence interval, 5-20 months) in the 21 cases with follow-up data. 10 The most common recurrent organ was the liver, followed by lymph nodes, lungs, and bones. 9 The effectiveness of adjuvant and neoadjuvant chemotherapies is still difficult to assess because there are few treatment cases.…”

“…9 The effectiveness of adjuvant and neoadjuvant chemotherapies is still difficult to assess because there are few treatment cases. [10][11][12][13]19,20 In summary, NECs of the extrahepatic bile duct are extremely rare and difficult to diagnose preoperatively. In addition, this disease can progress rapidly after surgical resection and has a poor prognosis.…”

Primary extrahepatic biliary neuroendocrine tumor: a case report

“…Ki-67 index is believed to be the most helpful in establishing the diagnosis with rates of <2% portending good long-term survival [ 3 ]. Additionally complete pathologic review of the specimen assists in ruling out mixed or composite tumor with non-neuroendocrine components which can suggest a more aggressive nature [ 9 , 10 ].…”

Case of resected small-cell neuroendocrine carcinoma of the extrahepatic bile duct

“…Kamiya et al . reported 25 resected cases of EHBD NEC [ 5 ]. Similar to our case, most of these patients presented with obstructive jaundice as the primary symptom and were preoperatively diagnosed with EHBD cancer.…”