Primary Hepatic Extra-gastrointestinal Stromal Tumors: Molecular Pathogenesis, Immunohistopathology, and Treatment

ABSTRACT: Gastrointestinal stromal tumors are the most common mesenchymal tumors of the gastrointestinal tract. They originate from the interstitial cells of Cajal and are usually found in extrahepatic gastrointestinal sites. However, a small subset are derived from the liver and are known as primary hepatic gastrointestinal stromal tumors (PHGIST). They have a poor prognosis and are historically difficult to diagnose. Our objective was to review and update the latest evidence-based knowledge concerning PHGIST, with a focus on epidemiology, etiology, pathophysiology, clinical presentation, histopathology, and treatment. These tumors are usually found incidentally, occur sporadically, and are associated with mutations of KIT and PDGFRA genes. PHGIST is a diagnosis of exclusion, as it has the same mol

SUBMITTER: Becker E 

PROVIDER: S-EPMC10318283 | biostudies-literature | 2023 Jan

REPOSITORIES: biostudies-literature