Published online Oct 28, 2020. doi: 10.3748/wjg.v26.i40.6195
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
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.
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.