<i>TFE3</i> -rearranged PEComa-like neoplasm harboring a novel <i>ZBED6::TFE3</i> fusion with an unusual immunophenotype

Based on a literature search of PubMed using the terms "PEComa", "PEComa-like", and "TFE3", this is the first case of a TFE3-rearranged PEComa-like neoplasm with ZBED6::TFE3 fusion, along with unusual morphologic and immunohistochemical phenotypes. The identification of the current case further expands the pathologic and genetic spectrum of this rare entity, highlighting the importance of integrating morphologic, immunohistochemical, and molecular findings in such cases.

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Publication Details

Journal
American Journal of Clinical Pathology
Published
2026-03-01
DOI
https://doi.org/10.1093/ajcp/aqag017
Primary Topic
Tuberous Sclerosis Complex Research
Type
article
Field-Weighted Citation Impact
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article

TFE3 -rearranged PEComa-like neoplasm harboring a novel ZBED6::TFE3 fusion with an unusual immunophenotype

Xia He, Hongying Zhang, Ran Peng, Wenyi Jing et al.
American Journal of Clinical Pathology
Tuberous Sclerosis Complex Research
article

TFE3 -rearranged PEComa-like neoplasm harboring a novel ZBED6::TFE3 fusion with an unusual immunophenotype

Xia He, Hongying Zhang, Ran Peng, Wenyi Jing, Lijuan Cui, Min Chen, Yue Wu
article en

Abstract

Based on a literature search of PubMed using the terms "PEComa", "PEComa-like", and "TFE3", this is the first case of a TFE3-rearranged PEComa-like neoplasm with ZBED6::TFE3 fusion, along with unusual morphologic and immunohistochemical phenotypes. The identification of the current case further expands the pathologic and genetic spectrum of this rare entity, highlighting the importance of integrating morphologic, immunohistochemical, and molecular findings in such cases.

American Journal of Clinical PathologyVol. 165(3)
Sichuan University (CN), West China Hospital of Sichuan University (CN), Suizhou Central Hospital (CN)
Gender equality
Openalex Percentile: Top 4%
Tuberous Sclerosis Complex Research
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