Zheng BQ, Wang ZP, Feng RZ, Zheng ZC, Wang HJ, Wang H, Tuo HF. Large cell neuroendocrine carcinoma of the distal common bile duct: A case report and literature review. World J Clin Cases 2026; 14(24): 123303 [DOI: 10.12998/wjcc.123303]
Corresponding Author of This Article
Hong-Fang Tuo, Associate Professor, Second Department of Hepatobiliary and Pancreatic Surgery, Hebei Provincial People’s Hospital, No. 348 Heping West Road, Xinhua District, Shijiazhuang 050051, Hebei Province, China. tuohongfang2023@163.com
Research Domain of This Article
Oncology
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case-report
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Zheng BQ, Wang ZP, Feng RZ, Zheng ZC, Wang HJ, Wang H, Tuo HF. Large cell neuroendocrine carcinoma of the distal common bile duct: A case report and literature review. World J Clin Cases 2026; 14(24): 123303 [DOI: 10.12998/wjcc.123303]
Bo-Qian Zheng, Ze-Cun Zheng, Hao Wang, Graduate School, Hebei Medical University, Shijiazhuang 050051, Hebei Province, China
Ze-Pu Wang, Department of Hepatobiliary and Pancreatic Surgery II, Hebei General Hospital, Shijiazhuang 050051, Hebei Province, China
Rui-Zeng Feng, Hong-Jie Wang, Graduate School, Hebei North University, Zhangjiakou 075000, Hebei Province, China
Hong-Fang Tuo, Second Department of Hepatobiliary and Pancreatic Surgery, Hebei Provincial People’s Hospital, Shijiazhuang 050051, Hebei Province, China
Author contributions: Wang ZP was responsible for manuscript drafting, data collection, and literature review; Zheng BQ performed pathological analysis and interpretation and contributed to manuscript drafting; Tuo HF was responsible for study conception, supervision, and final approval; Wang H, Feng RZ, and Zheng ZC contributed to the supplementation of key references; Wang HJ prepared the figures; all authors have read and approved the final manuscript.
AI contribution statement: AI tools were not used for any part of the scientific content, analysis, or writing of this manuscript. The authors assume full responsibility for the integrity, accuracy, and originality of the work.
Informed consent statement: Written informed consent was obtained from the patient for the publication of this case report and the accompanying images.
Conflict-of-interest statement: All authors declare that they have no conflicts of interest to disclose.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised in accordance with the CARE Checklist (2016).
Corresponding author: Hong-Fang Tuo, Associate Professor, Second Department of Hepatobiliary and Pancreatic Surgery, Hebei Provincial People’s Hospital, No. 348 Heping West Road, Xinhua District, Shijiazhuang 050051, Hebei Province, China. tuohongfang2023@163.com
Received: May 18, 2026 Revised: June 18, 2026 Accepted: August 5, 2026 Published online: August 26, 2026 Processing time: 96 Days and 8.9 Hours
Abstract
BACKGROUND
Large cell neuroendocrine carcinoma (LCNEC) of the extrahepatic bile duct is an exceedingly rare and highly aggressive malignancy, accounting for < 0.2% of all extrahepatic bile duct tumors. Preoperative diagnosis is challenging, and early recurrence is common even after R0 resection. No standard treatment regimen has been established to date. Mismatch repair (MMR) status has important implications for immunotherapy indications; however, it has rarely been reported in biliary LCNEC.
CASE SUMMARY
A 73-year-old man presented with obstructive jaundice. Imaging revealed a distal common bile duct mass. After percutaneous transhepatic cholangial drainage for biliary decompression, laparoscopic pancreaticoduodenectomy was performed. Pathological analysis confirmed LCNEC with a minor adenocarcinoma component, positive bile duct margin, and proficient MMR (pMMR)/microsatellite stable (MSS) phenotype. The patient received six cycles of adjuvant etoposide plus (EP) cisplatin and remained progression-free at 12-months follow-up (April 25, 2026), with no evidence of recurrence on imaging.
CONCLUSION
Biliary LCNEC is a rare and aggressive tumor. The pMMR/MSS phenotype provides a molecular rationale for excluding immunotherapy and directs treatment toward platinum-based chemotherapy. Even with a positive margin (R1), individualized adjuvant EP chemotherapy can achieve short-term disease-free survival. Multidisciplinary-guided, molecularly driven individualized therapy is central to the management of such rare malignancies.
Core Tip: This case report describes a distal common bile duct large cell neuroendocrine carcinoma (LCNEC) with a positive bile duct margin (R1 resection). Comprehensive immunohistochemistry revealed a proficient mismatch repair (MMR)/microsatellite stable phenotype, providing a molecular basis for excluding postoperative adjuvant immunotherapy. This is the first report of MMR testing directly guiding treatment decisions in biliary LCNEC, and clinicians are advised to incorporate MMR testing as a routine examination for biliary neuroendocrine carcinoma to inform immunotherapy decisions. With effective systemic therapy, positive margin (R1) resection does not preclude the possibility of favorable short-term outcomes. Multidisciplinary management that integrates molecular pathology is key to individualized treatment of rare tumors.
