{"title":"Keratin-positive giant cell-rich tumor review","authors":"John S.A. Chrisinger , Carina A. Dehner","doi":"10.1016/j.semdp.2025.150939","DOIUrl":null,"url":null,"abstract":"<div><div>Keratin-positive giant cell-rich tumor is a recently described mesenchymal neoplasm, which occurs predominantly in young women and often arises in the subcutis or bone. Histologically, tumors vary from giant cell tumor-like to xanthogranulomatous, or a mixture of both patterns. Tumors with predominantly xanthogranulomatous infiltrate and scattered mononuclear cells with bright eosinophilic cytoplasm were originally described as xanthogranulomatous epithelial tumor, a lesion which was subsequently found to be on a morphologic spectrum with keratin-positive giant cell-rich tumor. Most cases express keratin, characteristically with demonstration of dendritic-like cytoplasmic projections, and harbor HMGA2::NCOR2 fusions. High level expression of CSF1, in the absence of CSF1 gene alterations, is also frequently observed. Data on the clinical behavior of keratin-positive giant cell-rich tumor is limited. The course is often indolent, however rare cases are aggressive.</div></div>","PeriodicalId":49548,"journal":{"name":"Seminars in Diagnostic Pathology","volume":"42 5","pages":"Article 150939"},"PeriodicalIF":3.5000,"publicationDate":"2025-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Seminars in Diagnostic Pathology","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S0740257025000759","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"MEDICAL LABORATORY TECHNOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Keratin-positive giant cell-rich tumor is a recently described mesenchymal neoplasm, which occurs predominantly in young women and often arises in the subcutis or bone. Histologically, tumors vary from giant cell tumor-like to xanthogranulomatous, or a mixture of both patterns. Tumors with predominantly xanthogranulomatous infiltrate and scattered mononuclear cells with bright eosinophilic cytoplasm were originally described as xanthogranulomatous epithelial tumor, a lesion which was subsequently found to be on a morphologic spectrum with keratin-positive giant cell-rich tumor. Most cases express keratin, characteristically with demonstration of dendritic-like cytoplasmic projections, and harbor HMGA2::NCOR2 fusions. High level expression of CSF1, in the absence of CSF1 gene alterations, is also frequently observed. Data on the clinical behavior of keratin-positive giant cell-rich tumor is limited. The course is often indolent, however rare cases are aggressive.
期刊介绍:
Each issue of Seminars in Diagnostic Pathology offers current, authoritative reviews of topics in diagnostic anatomic pathology. The Seminars is of interest to pathologists, clinical investigators and physicians in practice.