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World J Gastroenterol. Oct 28, 2020; 26(40): 6195-6206
Published online Oct 28, 2020. doi: 10.3748/wjg.v26.i40.6195
Prevalence, diagnosis, and treatment of primary hepatic gastrointestinal stromal tumors
Xiao-Hui Qian, Ying-Cai Yan, Bing-Qiang Gao, Wei-Lin Wang
Xiao-Hui Qian, Ying-Cai Yan, Bing-Qiang Gao, Wei-Lin Wang, Department of Hepatobiliary and Pancreatic Surgery, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310009, Zhejiang Province, China
Xiao-Hui Qian, Bing-Qiang Gao, Wei-Lin Wang, Key Laboratory of Precision Diagnosis and Treatment for Hepatobiliary and Pancreatic Tumor of Zhejiang Province, Hangzhou 310009, Zhejiang Province, China
Xiao-Hui Qian, Bing-Qiang Gao, Wei-Lin Wang, Research Center of Diagnosis and Treatment Technology for Hepatocellular Carcinoma of Zhejiang Province, Hangzhou 310009, Zhejiang Province, China
Xiao-Hui Qian, Bing-Qiang Gao, Wei-Lin Wang, Clinical Medicine Innovation Center of Precision Diagnosis and Treatment for Hepatobiliary and Pancreatic Disease of Zhejiang University, Hangzhou 310009, Zhejiang Province, China
Xiao-Hui Qian, Bing-Qiang Gao, Wei-Lin Wang, Clinical Research Center of Hepatobiliary and Pancreatic Diseases of Zhejiang Province, Hangzhou 310009, Zhejiang Province, China
Author contributions: Qian XH designed the study, prepared the table, and wrote the paper; Yan YC and Gao BQ performed the research and analyzed and interpreted the data; Wang WL critically reviewed and drafted the manuscript, supervised, and finally approved the article.
Supported by National Natural Science Foundation of China, No. 81572307 and 81773096.
Conflict-of-interest statement: The authors have no conflicts of interest to declare.
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: Wei-Lin Wang, MD, PhD, Doctor, Professor, Surgeon, Department of Hepatobiliary and Pancreatic Surgery, The Second Affiliated Hospital, Zhejiang University School of Medicine, No. 88 Jiefang Road, Hangzhou 310009, Zhejiang Province, China. wam@zju.edu.cn
Received: May 13, 2020
Peer-review started: May 13, 2020
First decision: May 21, 2020
Revised: May 27, 2020
Accepted: September 22, 2020
Article in press: September 22, 2020
Published online: October 28, 2020
Processing time: 168 Days and 1.1 Hours
Abstract

Gastrointestinal stromal tumors (GIST), which is the most common mesenchymal tumor of the digestive tract, account for 1%-3% of gastrointestinal tumors. Primary stromal tumors outside the gastrointestinal tract are collectively referred to as extra GISTs, and stromal tumors in different regions often have different prognoses. A primary hepatic GIST is a rare tumor with an unknown origin, which may be related to interstitial Cajal-like cells. Although primary hepatic GIST has certain characteristics on imaging, it lacks specific symptoms and signs; thus, the final diagnosis depends on pathological and genetic evidence. This review summarizes all cases of primary hepatic GIST described in the literature and comprehensively analyzes the detailed clinical data of all patients. In terms of treatment, local resection alone or with adjuvant therapy was the prioritized choice to obtain better disease-free survival and longer survival time. For advanced unresectable cases, imatinib mesylate was applied as the first-line chemotherapy agent. Moreover, transcatheter arterial chemoembolization, radiofrequency ablation, and microwave ablation were shown to improve overall survival for selected patients. Liver transplantation was a final treatment option after resistance to chemotherapy developed.

Keywords: Extra gastrointestinal stromal tumor; Treatment; Liver transplant; Review

Core Tip: A primary hepatic gastrointestinal stromal tumor(PHGIST) is a rare tumor with an unknown origin and bad prognosis, which has always been misdiagnosed. The majority of studies of PHGIST are case reports, and clinical evaluation of different treatment methods has never been established. This review, including 35 cases from different nations, summarizes the etiology, clinical characteristics, diagnosis, treatment, and prognosis of PHGIST in order to clarify the treatment options for this rare disease.