Prevalence, diagnosis, and treatment of primary hepatic gastrointestinal stromal tumors

World J Gastroenterol. 2020 Oct 28;26(40):6195-6206. doi: 10.3748/wjg.v26.i40.6195.

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; Liver transplant; Review; Treatment.

Publication types

  • Review

MeSH terms

  • Antineoplastic Agents* / therapeutic use
  • Benzamides / therapeutic use
  • Carcinoma, Hepatocellular*
  • Chemoembolization, Therapeutic*
  • Gastrointestinal Neoplasms* / diagnosis
  • Gastrointestinal Neoplasms* / epidemiology
  • Gastrointestinal Neoplasms* / therapy
  • Gastrointestinal Stromal Tumors* / diagnosis
  • Gastrointestinal Stromal Tumors* / epidemiology
  • Gastrointestinal Stromal Tumors* / therapy
  • Humans
  • Liver Neoplasms* / epidemiology
  • Liver Neoplasms* / therapy
  • Piperazines / therapeutic use
  • Prevalence
  • Pyrimidines

Substances

  • Antineoplastic Agents
  • Benzamides
  • Piperazines
  • Pyrimidines