Systematic Review
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Meta-Anal. May 31, 2019; 7(5): 224-233
Published online May 31, 2019. doi: 10.13105/wjma.v7.i5.224
Hepatic gastrointestinal stromal tumor: Systematic review of an exceptional location
Alba Manuel-Vázquez, Raquel Latorre-Fragua, Roberto de la Plaza-Llamas, José Manuel Ramia
Alba Manuel-Vázquez, Raquel Latorre-Fragua, Roberto de la Plaza-Llamas, José Manuel Ramia, General and Digestive Surgery, University Hospital of Guadalajara, Guadalajara 19002, Spain
Author contributions: Manuel-Vázquez A designed the study, performed the research, analyzed data and wrote the paper. Latorre-Fragua R performed the research, analyzed and interpreted of data. de la Plaza-Llamas R critically reviewed. Ramia JM critically reviewed, finally approved and supervised the report.
Conflict-of-interest statement: The authors have no conflict of interest to declare.
PRISMA 2009 Checklist statement: The authors have read the PRISMA 2009 Checklist, and the manuscript was prepared and revised according to the PRISMA 2009 Checklist.
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: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Alba Manuel Vázquez, MD, PhD, Doctor, Surgeon, Surgical Oncologist, General and Digestive Surgery, University Hospital of Guadalajara, Calle donante de sangre s/n, Guadalajara 19002, Spain. alba_manuel_vazquez@hotmail.com
Telephone: +34-949-209200
Received: April 3, 2019
Peer-review started: April 3, 2019
First decision: April 30, 2019
Revised: May 11, 2019
Accepted: May 22, 2019
Article in press: May 26, 2019
Published online: May 31, 2019
Processing time: 58 Days and 20.9 Hours
Abstract
BACKGROUND

A minor subset of primary gastrointestinal stromal tumors (GIST) can also arise outside the gastrointestinal tract, which is known as an extra-GIST (E-GIST). Primary GIST of the liver is an exceptional location.

AIM

To characterize epidemiological, clinical and pathological features and options of treatments.

METHODS

We performed a systematic review to search for articles on primary hepatic GIST.

RESULTS

This review shows that right hepatic lobe was the most frequent location. Regarding pathological and immunohistochemical features, mitotic count was ≥ 5/50 High Power Fields in more than 50%; and CD117 was negative in only 1 patient. More than 70% of patients had a lesion with high risk of malignancy.

CONCLUSION

The diagnosis of E-GIST must be considered in a liver mass. Rendering an accurate diagnosis is a challenge, as well as the confirmation of their primary or metastatic nature.

Keywords: Gastrointestinal stromal tumors; Extra-gastrointestinal stromal tumor; Primary hepatic tumor; CD117; Primary hepatic gastrointestinal stromal tumor; Primary gastrointestinal stromal tumor of the liver

Core tip: A great majority of primary gastrointestinal stromal tumors (GISTs) outside the gastrointestinal tract (GI) are metastases; however, a minor subset of primary GISTs can also arise outside the GI tract which is known as an extra-GIST (E-GIST). Among E-GIST, liver is an exceptional location. We systematically review the literature on primary GIST of the liver. Primary hepatic EGISTs have a male predominance and usually are incidental findings. The surgical approach is commonly performed, and the final diagnosis is made by pathological, immunohistochemical and molecular analysis. Primary hepatic EGISTs are often high-risk lesions. Literature is scarce and it is very difficult to establish guidelines for clinicians.