TFE3‐rearranged nonmelanotic renal PEComa: a case series expanding their phenotypic and fusion landscape

This study highlights the morphologic and genetic diversity of TFE3-rearranged primary renal PEComas and underlines the value of surrogate TFE3 immunohistochemistry in identifying them. The lack of PAX8 and keratin expression represents the mainstay for distinguishing these tumours from MiTF-associated renal cell carcinomas. In addition, we report rare (ZC3H4, RBMX) and novel (MED15) TFE3 fusion partners in PEComa.

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Publication Details

Journal
Histopathology
Published
2024-08-21
DOI
https://doi.org/10.1111/his.15304
Citations
16
Primary Topic
Tuberous Sclerosis Complex Research
Type
article
Field-Weighted Citation Impact
5.22
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article

TFE3‐rearranged nonmelanotic renal PEComa: a case series expanding their phenotypic and fusion landscape

Sean R. Williamson, Eddie Fridman, Kiril Trpkov, Christoph Schubart et al.
16 citations
Histopathology
Tuberous Sclerosis Complex Research
5.22
article

TFE3‐rearranged nonmelanotic renal PEComa: a case series expanding their phenotypic and fusion landscape

Sean R. Williamson, Eddie Fridman, Kiril Trpkov, Christoph Schubart, Abbas Agaimy, Andres Martin Acosta, Katrina Collins, Liang Cheng, Arndt Hartmann
article en
16 citations

Abstract

This study highlights the morphologic and genetic diversity of TFE3-rearranged primary renal PEComas and underlines the value of surrogate TFE3 immunohistochemistry in identifying them. The lack of PAX8 and keratin expression represents the mainstay for distinguishing these tumours from MiTF-associated renal cell carcinomas. In addition, we report rare (ZC3H4, RBMX) and novel (MED15) TFE3 fusion partners in PEComa.

HistopathologyVol. 85(5)
Cleveland Clinic (US), Tel Aviv University (IL), University of Calgary (CA), Friedrich-Alexander-Universität Erlangen-Nürnberg (DE), Brown University (US), Sheba Medical Center (IL), Calgary Laboratory Services (CA), Comprehensive Cancer Center Erlangen (DE), Indiana University School of Medicine, Indiana University – Purdue University Indianapolis (US)
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Openalex Percentile: Top 4%
Tuberous Sclerosis Complex Research
5.22
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