Retrospective Study
Copyright ©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Oncol. Sep 15, 2020; 12(9): 1031-1043
Published online Sep 15, 2020. doi: 10.4251/wjgo.v12.i9.1031
Clinical characteristics and outcome of primary hepatic neuroendocrine tumors after comprehensive therapy
Hao-Hao Wang, Zhao-Chen Liu, Gong Zhang, Lu-Hao Li, Lin Li, Qing-Bo Meng, Pei-Ju Wang, Dong-Qi Shen, Xiao-Wei Dang
Hao-Hao Wang, Zhao-Chen Liu, Gong Zhang, Lu-Hao Li, Lin Li, Qing-Bo Meng, Pei-Ju Wang, Dong-Qi Shen, Xiao-Wei Dang, Department of Hepatopancreatobiliary Surgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou 450000, Henan Province, China
Author contributions: Dang XW designed the research study; Meng QB, Wang PJ, and Shen DQ collected the information of patients; Zhang G, Li LH, and Li L performed the analysis of data; Wang HH and Liu ZC edited the final manuscript; and all authors read and approved the final manuscript.
Institutional review board statement: This study was approved by the Ethics Committees of the First Affiliated Hospital of Zhengzhou University.
Informed consent statement: Informed consent was obtained from all patients.
Conflict-of-interest statement: The authors declare that they have no conflict of interest to disclose.
Data sharing statement: No additional data are available.
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: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Xiao-Wei Dang, MD, Doctor, Surgeon, Department of Hepatopancreatobiliary Surgery, The First Affiliated Hospital of Zhengzhou University, No. 1 East Jianshe Road, Zhengzhou 450000, Henan Province, China. dangxiaowei1001@163.com
Received: April 8, 2020
Peer-review started: April 8, 2020
First decision: May 5, 2020
Revised: May 13, 2020
Accepted: August 15, 2020
Article in press: August 15, 2020
Published online: September 15, 2020
Abstract
BACKGROUND

Primary hepatic neuroendocrine tumors (PHNETs), a group of neuroendocrine neoplasms, are extremely rare. There are only few case reports about PHNETs in the literature. The lack of large samples and multicenter research results in poor diagnostic and therapeutic approaches.

AIM

To discuss the clinical characteristics, diagnosis, and treatment of PHNETs and risk factors related to survival.

METHODS

We retrospectively analyzed the clinical data, imaging features, immunohistochemistry data, and treatment efficacy of 40 patients who were pathologically diagnosed with PHNETs and admitted to The First Affiliated Hospital of Zhengzhou University from January 1, 2014 to November 15, 2019. Finally, survival analysis was performed to identify the risk factors for survival.

RESULTS

The main symptoms and signs included intermittent abdominal pain (19 patients, 47.5%) and bloating (8 patients, 20.0%). The positive rates of tested tumor markers were recorded as follows: Carbohydrate antigen 19-9 (CA19-9) (6 patients, 15.0%), CA72-4 (3 patients, 7.5%), carcinoembryonic antigen (7 patients, 17.5%), and alpha-fetoprotein (6 patients, 15.0%). Immunohistochemical staining results showed positivity for Syn in 38 (97.4%) of 39 patients, for chromogranin A in 17 (65.4%) of 26 patients, for CD56 in 35 (94.6%) of 37 patients, for AE1/AE3 in 28 (87.5%) of 32 patients, and for Ki-67 in all 40 (100.0%) patients. The overall survival rate was significantly related to the tumor grade, AE1/AE3, and Ki-67. No significant correlation was found between other parameters (age, gender, tumor number, tumor size, metastasis, and treatment) and overall survival.

CONCLUSION

Higher grade, negative AE1/AE3, and higher Ki-67 are associated with a worse survival rate. Kinds of treatment and other parameters have no significant influence on overall survival.

Keywords: Neuroendocrine tumors, Primary hepatic neuroendocrine tumors, Diagnosis, Survival analysis, Tumor grade, Treatment

Core Tip: Neuroendocrine tumors (NETs) are originally mainly from the gastrointestinal and bronchopulmonary tracts. Liver neuroendocrine tumors mostly metastasize from other organs, and primary hepatic NETs (PHNETs), which were first reported by Edmonson in 1958, are extremely rare and account for only 0.3% of all NETs. Furthermore, there are less than 150 PHNET cases described in English-language articles because of the low incidence. The longer-term, larger sample multi-center studies are urgently needed to figure out their clinicopathological features and prognostic factors, and finally facilitate their diagnosis and treatment.