ArticleVirchows Archiv : an international journal of pathology2025
YAP1::TFE3 fusion in a case of malignant TFE3-rearranged PEComa of the lung: expanding the spectrum of pulmonary PEComa-like mesenchymal neoplasms.
Article in Virchows Archiv : an international journal of pathology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.
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Who cites it
5 citing papers in PubMed.
- YAP1::TFE3-rearranged inflammatory spindle cell PEComa of the anterior mediastinum in a 5-year-old child: first pediatric case.Virchows Archiv : an international journal of pathology · 2026Article
- YAP1::TFE3 mediates endothelial-to-mesenchymal plasticity in epithelioid hemangioendothelioma.Molecular oncology · 2026Article
- Perivascular Epithelioid Cell Neoplasm of the Lung: A Patient Case Report.Case reports in pathology · 2026Article
- Clinicopathological and molecular features of gynecologic perivascular epithelioid cell tumors: a single-center study.Frontiers in oncology · 2026Article
- "Putting the cart before the horse": an update on promiscuous gene fusions in soft tissue tumors.Virchows Archiv : an international journal of pathology · 2025Review
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Authors and funding
4 authors.
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Abstract
The family of PEComa encompasses a heterogeneous group of related mesenchymal neoplasms with myomelanocytic differentiation, a distinctive subset of which is characterized by TFE3 gene rearrangement. Recurrent YAP1::TFE3 fusion has been found in clear cell stromal tumor of the lung (CCST-L), and most recently, in two cases classified as inflammatory spindle cell PEComa. However, the potential relationship between CCST-L and PEComa remains unclear. Herein, we report a case of primary pulmonary malignant TFE3-rearranged PEComa with prototypical morphological and immunohistochemical features, unexpectedly harboring YAP1::TFE3 fusion. Our findings further expanded the morphological and molecular spectrum of PEComa-like mesenchymal neoplasms of the lung.
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