{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,25]],"date-time":"2026-08-25T09:21:11Z","timestamp":1787649671896,"version":"build-2736575974"},"reference-count":42,"publisher":"SAGE Publications","license":[{"start":{"date-parts":[[2026,7,29]],"date-time":"2026-07-29T00:00:00Z","timestamp":1785283200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Int J Surg Pathol"],"abstract":"<jats:p>\n                    We report a primary perivascular epithelioid cell tumor (PEComa) of the kidney harboring a novel\n                    <jats:italic toggle=\"yes\">CLTC::TFE3<\/jats:italic>\n                    gene fusion in a 51-year-old woman. The tumor was composed of high-grade epithelioid cells with clear to eosinophilic cytoplasm arranged in nested and alveolar architectures, closely mimicking clear cell renal cell carcinoma, which posed a significant diagnostic pitfall. Immunophenotypically, the tumor lacked multiple epithelial and renal lineage markers but showed diffuse positivity for melanocytic markers and cathepsin K.\n                    <jats:italic toggle=\"yes\">TFE3<\/jats:italic>\n                    rearrangement was confirmed by fluorescence in situ hybridization and an in-frame\n                    <jats:italic toggle=\"yes\">CLTC::TFE3<\/jats:italic>\n                    fusion involving exons 1\u201317 of\n                    <jats:italic toggle=\"yes\">CLTC<\/jats:italic>\n                    and exons 6\u201310 of\n                    <jats:italic toggle=\"yes\">TFE3<\/jats:italic>\n                    was identified by subsequent RNA sequencing. The patient developed an aggressive clinical course, with early brain metastasis and multiple recurrences over a five-year period despite multiple radical surgical resections, radiotherapy, and systemic therapy. This tumor represents the first reported primary renal PEComa harboring a\n                    <jats:italic toggle=\"yes\">CLTC::TFE3<\/jats:italic>\n                    fusion and the second tumor with this fusion reported in the literature. Our findings expand the molecular spectrum of\n                    <jats:italic toggle=\"yes\">TFE3<\/jats:italic>\n                    -rearranged PEComa and underscore the importance of comprehensive immunohistochemical and molecular profiling in accurately diagnosing these rare tumors and distinguishing from their morphologic mimics.\n                  <\/jats:p>","DOI":"10.1177\/10668969261463689","type":"journal-article","created":{"date-parts":[[2026,7,29]],"date-time":"2026-07-29T13:03:24Z","timestamp":1785330204000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":0,"title":["Primary Renal Perivascular Epithelioid Cell Tumor with\n                    <i>CLTC::TFE3<\/i>\n                    Gene Fusion: A Rare Gene Fusion and Masquerading as Renal Cell Carcinoma"],"prefix":"10.1177","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4480-9146","authenticated-orcid":false,"given":"Geethanjali","family":"Gude","sequence":"first","affiliation":[{"name":"University of Texas Southwestern Medical Center"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-9029-5001","authenticated-orcid":false,"given":"Hilda","family":"Mirbaha","sequence":"additional","affiliation":[{"name":"University of Texas Southwestern Medical Center"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2118-6428","authenticated-orcid":false,"given":"Liwei","family":"Jia","sequence":"additional","affiliation":[{"name":"University of Texas Southwestern Medical 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