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
Case characteristics

The female patient suffered a giant but slow-growing, mild symptom right hepatic tumor for 26 years before definitive surgery, and the diagnosis was confirmed as primary hepatic neuroendocrine tumor (PHNET). Surgical removal leads to a good long-term disease free survival.

Clinical diagnosis

The female patient had no obvious symptoms but abdominal distension and intermittent abdominal pain.

Differential diagnosis

In the first surgery in 1984, tumor biopsy was performed and three differential diagnoses were considered: (1) Hepatocellular adenoma; (2) highly differentiated hepatocellular carcinoma; and (3) sinusoidal dilatation and hemorrhage.

Laboratory diagnosis

No abnormal findings were found in preoperative laboratory tests, including blood regular test, liver and renal function, tumor markers, etc.

Imaging diagnosis

Computed tomography scan showed a huge space-occupying lesion of the right liver, with the heterogeneous internal density and multiple enveloped calcifications.

Pathological diagnosis

Histological and immunochemical exams confirmed acinar and daisy clumpy structures under light microscope. Some areas were beam-like and had rich sinusoids, and mitotic phases were rare (0-1/10 HPF). Cluster of differentiation 56 and synaptophysin were positive, and Ki-67 was < 1% in the tumor.


Patient accepts definitive surgery, and the tumor was removed thoroughly.

Related reports

PHNET is a kind of very rare primary hepatic lesion. The symptoms, radiological findings, clinical course, therapy methods, and patients’ survival conditions are variable between different cases. Although PHNET should be a differential diagnosis in liver tumors, it is very difficult to confirm this diagnosis before pathological examination. Different therapy methods have been used to treat PHNET. Surgery should be the first choice if possible.

Term explanation

Neuroendocrine Tumor (NET), previously named carcinoid tumor, is a kind of rare tumor. NETs always originate from gastrointestinal tracts or bronchial lungs. NETs in liver are rare, and about 80% of them are metastasis tumors. PHNET is extremely rare, accounting for roughly 0.4% of all cases of NETs, and it is difficult to differentiate from other liver tumors, such as hepatocellular carcinoma and adenoma.

Experiences and lessons

In this rare, slow growing PHNET case, we find that methods and the possibility of treatment may change along with technology progress, such that definitive treatment may be achieved after long-time conservative treatment.