Case Report
Copyright ©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Jun 28, 2018; 24(24): 2640-2646
Published online Jun 28, 2018. doi: 10.3748/wjg.v24.i24.2640
Primary hepatic neuroendocrine tumor case with a preoperative course of 26 years: A case report and literature review
Xiang-Fei Meng, Ying-Wei Pan, Zhan-Bo Wang, Wei-Dong Duan
Xiang-Fei Meng, Ying-Wei Pan, Wei-Dong Duan, Department of Hepatobiliary Surgery, Chinese PLA General Hospital, Beijing 100853, China
Zhan-Bo Wang, Department of Pathology, Chinese PLA General Hospital, Beijing 100853, China
Author contributions: Meng XF attended the patient’s therapy and follow-up, analyzed the data, and wrote the paper; Pan YW collected the patient’s data; Wang ZB gave the detailed pathological analysis; Duan WD was responsible for the patient’s therapy, follow-up, and revised the paper.
Informed consent statement: The patient involved in this study gave her written informed consent authorizing use and disclosure of her protected health information.
Conflict-of-interest statement: All the authors have no conflicts of interests to declare.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (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:
Correspondence to: Wei-Dong Duan, MD, PhD, Associated Professor, Attending Doctor, Surgeon, Department of Hepatobiliary Surgery, Chinese PLA General Hospital, 28th FuXing Road, HaiDian District, Beijing 100853, China.
Telephone: +86-10-66938334
Received: March 22, 2018
Peer-review started: March 22, 2018
First decision: April 24, 2018
Revised: May 12, 2018
Accepted: May 26, 2018
Article in press: May 26, 2018
Published online: June 28, 2018

Primary hepatic neuroendocrine tumor (PHNET) is an extremely rare liver tumor. Patients often have no clinical symptoms or have only non-specific symptoms, such as abdominal pain and abdominal mass. The clinical manifestations, disease development, treatment methods, and treatment outcomes of PHNET vary greatly among cases. Here we report a case of PHNET with a confirmed 26-year survival before surgery. The patient was a 56-year-old female. A large right hepatic mass was detected when the patient was 30 years old. The tumor could not be removed during exploratory laparotomy, and constriction of the right hepatic artery and biopsy were conducted. Pathological results indicated a diagnosis of benign tumor, but a confirmed diagnosis was not reached. Twenty-six years after the patient had been living with the tumor, she sought treatment again because of tumor progression. After systematic evaluation of the resectability, the tumor was resected. Based on the examination results of the gastrointestinal tract and lungs, intraoperative examination results, pathological findings, and long-term follow-up results, the diagnosis of PHNET was confirmed. This case represents the longest reported survival time for a PHNET patient before removal of the tumor.

Keywords: Primary hepatic neuroendocrine tumor, Surgery, Pathology, Radiology, Surgical plan, Follow up

Core tip: In this work, we reported a rare case of primary hepatic neuroendocrine tumor. To our knowledge, the total number of cases does not exceed 150. The diagnosis, radiological features, treatment, and survival conditions vary among cases. Our case has two features that have not yet been reported in the literature: (1) The patient had a confirmed preoperative disease course of 26 years-the longest preoperative course among the reported cases; and (2) the lesion contained multiple calcifications, which is not reported previously.