Case Report
Copyright ©2014 Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Dec 21, 2014; 20(47): 18048-18052
Published online Dec 21, 2014. doi: 10.3748/wjg.v20.i47.18048
Primary large cell neuroendocrine carcinoma in the common bile duct: First Asian case report
Sung Bae Park, Suk Bae Moon, Young Joon Ryu, Jeana Hong, Yang Hee Kim, Gi Bong Chae, Seong Kweon Hong
Sung Bae Park, Suk Bae Moon, Yang Hee Kim, Gi Bong Chae, Seong Kweon Hong, Department of Surgery, Kangwon National University Hospital, Kangwon National University School of Medicine, Chuncheon 200-947, South Korea
Young Joon Ryu, Department of Pathology, Kangwon National University Hospital, Kangwon National University School of Medicine, Chuncheon 200-947, South Korea
Jeana Hong, Department of Pediatrics, Kangwon National University Hospital, Kangwon National University School of Medicine, Chuncheon 200-947, South Korea
Author contributions: Park SB and Hong SK designed the report; Ryu YJ performed the pathologic review; Moon SB, Kim YH, Chae GB, and Hong J advised and wrote the paper.
Correspondence to: Seong Kweon Hong, MD, Director, Department of Surgery, Kangwon National University Hospital, Kangwon National University School of Medicine, 17-1 Hyoja 3 Dong, Kangwong-Do, Chuncheon 200-947, South Korea. skhong1@kangwon.ac.kr
Telephone: +82-33-2582288 Fax: +82-33-2582169
Received: August 25, 2014
Revised: October 7, 2014
Accepted: November 18, 2014
Published online: December 21, 2014
Processing time: 117 Days and 7.2 Hours
Abstract

Large cell neuroendocrine carcinoma (LCNEC) in the biliary system is a poorly differentiated, high-grade neuroendocrine tumor. These tumors exhibit aggressive behavior and an increased tendency for early nodal and distant metastases. Herein, we report an unusual case of a pure primary LCNEC of the common bile duct (CBD). A 75-year-old female presented with nausea and jaundice. The patient underwent a CBD excision with lymph node dissection. Upon histological and immunohistochemical examination, the tumor exhibited pure large cell-type neuroendocrine features. Metastases were noted in two of the eight lymph nodes. The patient was administered adjuvant chemotherapy. The patient’s cancer recurred 7 mo after surgery, and the patient died from liver failure 5 mo after recurrence. The prognosis of LCNEC of CBD remains poor despite curative resection and adjuvant chemotherapy. The role of additional therapies, such as multimodal treatment including radiation therapy, must be further studied to improve the prognoses of patients.

Keywords: Neuro endocrine tumor; Large cell neuroendocrine carcinoma; Metastases; Multimodal treatment; Common bile duct

Core tip: Primary neuroendocrine carcinomas of the common bile duct rarely occur; among these tumors, large cell neuroendocrine carcinoma is extremely rare. Large cell neuroendocrine carcinoma (LCNEC) is a high-grade malignant neuroendocrine neoplasm. This case report represents a rare case of a primary neuroendocrine carcinoma of the common bile duct. More clinicopathological data and further studies with multimodal treatment are required to identify the prognostic indicators and histogenesis of LCNEC of common bile duct.