{"title":"[Gastric tumors with GLI1 gene alterations (plexiform fibromyxoma and gastroblastoma). Case report and literature review].","authors":"I V Sidorov, A S Sharlai, D M Konovalov","doi":"10.17116/patol20258701137","DOIUrl":null,"url":null,"abstract":"<p><p>Plexiform fibromyxoma (PFM) and gastroblastoma (GB) are rare gastric tumors with a specific <i>MALAT1::GLI1</i> rearrangement, included in the conditional spectrum of neoplasms with alterations of the <i>GLI1</i> gene. The article presents a clinical case of PFM in a 6-year-old girl and a literature review highlighting current data on the morphology, immunophenotype and molecular genetic characteristics of PFM and GB. Despite the common genetic anomaly, differences in the morphology and clinical course of these tumors indicate the need for further research to clarify their relationship and potential reclassification in the light of new data on tumors with <i>GLI1</i> gene abnormalities. Integrating the accumulated knowledge about tumors with <i>GLI1</i> gene alterations into diagnostic algorithms and therapeutic approaches will help improve the treatment outcomes of patients with these rare neoplasms.</p>","PeriodicalId":8548,"journal":{"name":"Arkhiv patologii","volume":"87 1","pages":"37-40"},"PeriodicalIF":0.0000,"publicationDate":"2025-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Arkhiv patologii","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.17116/patol20258701137","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"Medicine","Score":null,"Total":0}
引用次数: 0
Abstract
Plexiform fibromyxoma (PFM) and gastroblastoma (GB) are rare gastric tumors with a specific MALAT1::GLI1 rearrangement, included in the conditional spectrum of neoplasms with alterations of the GLI1 gene. The article presents a clinical case of PFM in a 6-year-old girl and a literature review highlighting current data on the morphology, immunophenotype and molecular genetic characteristics of PFM and GB. Despite the common genetic anomaly, differences in the morphology and clinical course of these tumors indicate the need for further research to clarify their relationship and potential reclassification in the light of new data on tumors with GLI1 gene abnormalities. Integrating the accumulated knowledge about tumors with GLI1 gene alterations into diagnostic algorithms and therapeutic approaches will help improve the treatment outcomes of patients with these rare neoplasms.
期刊介绍:
The journal deals with original investigations on pressing problems of general pathology and pathologic anatomy, newest research methods, major issues of the theory and practice as well as problems of experimental, comparative and geographic pathology. To inform readers latest achievements of Russian and foreign medicine the journal regularly publishes editorial and survey articles, reviews of the most interesting Russian and foreign books on pathologic anatomy, new data on modern methods of investigation (histochemistry, electron microscopy, autoradiography, etc.), about problems of teaching, articles on the history of pathological anatomy development both in Russia and abroad.