{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,23]],"date-time":"2026-07-23T17:52:32Z","timestamp":1784829152732,"version":"3.55.0"},"reference-count":21,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2026,3,6]],"date-time":"2026-03-06T00:00:00Z","timestamp":1772755200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,3,6]],"date-time":"2026-03-06T00:00:00Z","timestamp":1772755200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100018935","name":"Universit\u00e4tsklinikum Erlangen","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100018935","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Virchows Arch"],"published-print":{"date-parts":[[2026,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms characterized by myomelanocytic differentiation and dichotomic molecular pathogenesis (mTOR-activating mutations versus\n                    <jats:italic>TFE3<\/jats:italic>\n                    gene rearrangements). A recently recognized subset harboring\n                    <jats:italic>YAP1::TFE3<\/jats:italic>\n                    gene fusion, termed inflammatory spindle cell PEComa, has been described predominantly in the lungs of adults showing significant morphologic and molecular overlap with\n                    <jats:italic>YAP1::TFE3<\/jats:italic>\n                    \u2013fused clear cell stromal tumor of the lung (CCST-L). We report the first pediatric case of\n                    <jats:italic>YAP1::TFE3<\/jats:italic>\n                    \u2013rearranged inflammatory spindle cell PEComa arising in the anterior mediastinum of a 5-year-old child. The tumor, largely enclosed by a thick fibrous capsule, was composed of plump ovoid to spindle cells with a prominent chronic mononuclear inflammatory infiltrate and strikingly extensive multifocal stromal dystrophic calcifications that were radiologically conspicuous. Worrisome features included large size (8\u00a0cm) and multifocal necrosis. Tumor cells showed diffuse expression of melanocytic markers (HMB45 and melanoma cocktail), cathepsin K and TFE3 in the absence of smooth muscle markers. The C-terminal YAP1 staining revealed complete loss limited to the neoplastic cells. This case expands the clinicopathologic and age spectrum of\n                    <jats:italic>YAP1::TFE3<\/jats:italic>\n                    \u2013rearranged PEComa which needs to be distinguished from other overlapping neoplasms carrying the same gene fusion.\n                  <\/jats:p>","DOI":"10.1007\/s00428-026-04470-w","type":"journal-article","created":{"date-parts":[[2026,3,6]],"date-time":"2026-03-06T04:38:37Z","timestamp":1772771917000},"page":"225-231","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["YAP1::TFE3\u2013rearranged inflammatory spindle cell PEComa of the anterior mediastinum in a 5-year-old child: first pediatric case"],"prefix":"10.1007","volume":"489","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5747-8900","authenticated-orcid":false,"given":"Rola H.","family":"Ali","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Doaa","family":"Hamdy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ahmed","family":"Nasr","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nisreen","family":"Khalifa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0445-8161","authenticated-orcid":false,"given":"Abbas","family":"Agaimy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,3,6]]},"reference":[{"key":"4470_CR1","doi-asserted-by":"publisher","first-page":"387","DOI":"10.1002\/path.2289","volume":"214","author":"CC Pan","year":"2008","unstructured":"Pan CC, Chung MY, Ng KF, Liu CY, Wang JS, Chai CY, Huang SH, Chen PC, Ho DM (2008) Constant allelic alteration on chromosome 16p (TSC2 gene) in perivascular epithelioid cell tumour (PEComa): genetic evidence for the relationship of PEComa with angiomyolipoma. 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The authors have no financial or non-financial conflicts of interest to disclose.","order":2,"name":"Ethics","label":"Conflict of interest","group":{"name":"EthicsHeading","label":"Declarations"}}]}}