Gastrointestinal stromal tumors and second primary malignancies before and after the introduction of imatinib mesylate

Gastrointestinal stromal tumor （GIST） is the most common mesenchymal malignancy of the gastrointestinal tract.GISTs may coexist with different types of cancer,either synchronous or metachronous （1）.Most GISTs develop in a sporadic fashion,but familial occurrence,such as neurofibromatosis and Carney-triad,has also been reported （2）.The overall frequency of second tumors in different series varied from 4.5% to 33%.The most frequent types of GIST-associated cancers were gastrointestinal carcinomas （47%）,lymphoma/leukemia （7%）,carcinomas of prostate （9%）,breast （7%）,kidney （6%）,lung （5%）,female genital tract （5%）,carcinoid tumors （3%）,soft tissue and bone sarcomas （3%）,malignant melanoma （2%） and seminoma （1%） （1,3-5）.

Giant hepatic extra-gastrointestinal stromal tumor treated with cytoreductive surgery and adjuvant systemic therapy:A case report and review of literature

BACKGROUND Primary extra-gastrointestinal stromal tumors(E-GIST)of the liver are rare.The clinical presentation may range from asymptomatic to bleeding or manifestations of mass effect.Oncologic surgery followed by adjuvant therapy with imatinib is the standard of care.However,under specific circumstances,a cytoreductive approach may represent a therapeutic option.We describe herein the case of an 84-year-old woman who presented with a tender,protruding epigastric mass.Abdominal computed tomography scan revealed a large,heterogeneous mass located across segments III,IV,V,and VIII of the liver.The initial approach was transarterial embolization of the tumor,which elicited no appreciable response.Considering the large size and central location of the tumor and the advanced age of the patient,non-anatomic complete resection was indicated.Due to substantial intraoperative bleeding and hemodynamic instability,only a near-complete resection could be achieved.Histopathology and immunohistochemical staining confirmed the diagnosis of primary E-GIST of the liver.Considering the risk/benefit ratio for therapeutic options,debulking surgery may represent a strategy to control pain and prolong survival.CASE SUMMARY Here,we present a case report of a patient diagnosed with E-GIST primary of the liver,which was indicated a cytoreductive surgery and adjuvant therapy with imatinib.CONCLUSION E-GIST primary of the liver is a rare conditional,the treatment is with systemic therapy and total resection surgery.However,a cytoreductive surgery will be necessary when a complete resection is no possible.

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

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 molecular,immunochemistry and histological appearance as gastrointestinal stromal tumors(GIST).Thus,imaging,such as positron emission tomography-computed tomography(PET-CT)must be used to rule out metastatic GIST before a diagnosis can be made.However,with mutation analysis and pharmacological advances,tyrosine kinase inhibitors are typically pursued with or without surgical intervention.Other potential treatments include transcatheter arterial chemoembolization and tumor ablation.However,these are typically considered palliative options.As there are only a limited number of publications regarding PHGIST,data concerning morbidity and mortality are not yet available.Immunohistopathology can help develop screening guidelines and evaluating resistance to treatment.