Case Report
Copyright ©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Surg. May 27, 2024; 16(5): 1449-1460
Published online May 27, 2024. doi: 10.4240/wjgs.v16.i5.1449
Neuroendocrine carcinoma of the common hepatic duct coexisting with distal cholangiocarcinoma: A case report and review of literature
Fei Chen, Wei-Wei Li, Juan-Fen Mo, Min-Jie Chen, Su-Hang Wang, Shu-Ying Yang, Zheng-Wei Song
Fei Chen, Min-Jie Chen, Zheng-Wei Song, Department of Hepatobiliary Surgery, The Second Affiliated Hospital of Jiaxing University, Jiaxing 314000, Zhejiang Province, China
Wei-Wei Li, Graduate School, Bengbu Medical College, Bengbu 233000, Anhui Province, China
Juan-Fen Mo, The Key Laboratory, The Second Affiliated Hospital of Jiaxing University, Jiaxing 314000, Zhejiang Province, China
Su-Hang Wang, Department of Pathology, The Second Affiliated Hospital of Jiaxing University, Jiaxing 314000, Zhejiang Province, China
Shu-Ying Yang, Department of Intensive Medicine, The Second Affiliated Hospital of Jiaxing University, Jiaxing 314000, Zhejiang Province, China
Co-first authors: Fei Chen and Wei-Wei Li.
Co-corresponding authors: Shu-Ying Yang and Zheng-Wei Song.
Author contributions: Chen F, Li WW, Mo JF, Yang SY, and Song ZW designed the research; Chen F, Li WW, and Mo JF performed the research; Chen MJ and Wang SH contributed new reagents/analytic tools; Chen F, Li WW, and Mo JF analyzed the data; Chen F, Li WW, Mo JF, and Yang SY wrote the paper. Chen F and Li WW contributed equally and shared co-first authorship. Yang SY and Song ZW contributed equally and shared corresponding author.
Supported by Medical Health Science and Technology Project of Zhejiang Provincial Health Commission, No. 2022KY1246; and Science and Technology Bureau of Jiaxing City, No. 2023AZ31002 and No. 2022AZ10009.
Informed consent statement: The patient provided their written informed consent to participate in this study. Written informed consent was obtained from the individual for the publication of any potentially identifiable images or data included in this article. The patient agreed to participate in this study and to publish this report.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Zheng-Wei Song, Doctor, Department of Hepatobiliary Surgery, The Second Affiliated Hospital of Jiaxing University, No. 1518 Huanchen North Road, Jiaxing 314000, Zhejiang Province, China. doctorsongzw@163.com
Received: January 27, 2024
Revised: March 13, 2024
Accepted: April 15, 2024
Published online: May 27, 2024
Processing time: 117 Days and 5.2 Hours
Abstract
BACKGROUND

Neuroendocrine carcinoma (NEC) of the extrahepatic bile duct is very rare, and the treatment and prognosis are unclear. Herein, we report the case of a middle-aged female with primary large cell NEC (LCNEC) of the common hepatic duct combined with distal cholangiocarcinoma (dCCA). Additionally, after a review of the relevant literature, we summarize and compare mixed neuroendocrine-non-neuroendocrine neoplasm (MiNEN) and pure NEC to provide a reference for selecting the appropriate treatment and predicting the prognosis of this rare disease.

CASE SUMMARY

A 62-year-old female presented to the hospital due to recurrent abdominal pain for 2 months. Physical examination showed mild tenderness in the upper abdomen and a positive Courvoisier sign. Blood tests showed elevated liver transaminase and carbohydrate antigen 199 levels. Imaging examination revealed a 1-cm tumour in the middle and lower segments of the common bile duct. Pancreaticoduodenectomy + lymph node dissection was performed, and hepatic duct tumours were unexpectedly found during surgery. Pathology suggested poorly differentiated LCNEC (approximately 0.5 cm × 0.5 cm × 0.4 cm), Ki-67 (50%), synaptophysin+, and chromogranin A+. dCCA pathology suggested moderately differentiated adenocarcinoma. The patient eventually developed lymph node metastasis in the liver, bone, peritoneum, and abdominal cavity and died 24 months after surgery. Gene sequencing methods were used to compare gene mutations in the two primary bile duct tumours.

CONCLUSION

The prognosis of MiNEN and pure NEC alone is different, and the selection of treatment options needs to be differentiated.

Keywords: Neuroendocrine carcinoma; Mixed neuroendocrine-non-neuroendocrine neoplasm; Cholangiocarcinoma; Extrahepatic bile duct; Case report

Core Tip: We reported a rare case of primary large cell neuroendocrine carcinoma (NEC) of the hepatic duct combined with distal cholangiocarcinoma (dCCA). The overall survival was 24 months, and the prognosis was relatively good, which may be related to the early stage and low Ki-67 index of NEC and the early stage and moderate differentiation of the coexisting dCCA. To explore genetic causes, gene sequencing was performed on the two types of cancer tissue. A total of 35 gene mutations were detected in NEC tissue, and 7 gene mutations were detected in adenocarcinoma tissue.