TFE3 activation in a TSC1-altered malignant PEComa: challenging the dichotomy of the underlying pathogenic mechanisms.

doi:10.1002/cjp2.187

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Article

TFE3 activation in a TSC1-altered malignant PEComa: challenging the dichotomy of the underlying pathogenic mechanisms.

The journal of pathology. Clinical research - 1 Jan 2021

Schmiester Maren, Dolnik Anna, Kornak Uwe, Pfitzner Berit, Hummel Michael, Treue Denise, Hartmann Arndt, Agaimy Abbas, Weyerer Veronika, Lekaj Anja, Brakemeier Susanne, Peters Robert, Öllinger Robert, Märdian Sven, Bullinger Lars, Striefler Jana Käthe, Flörcken Anne

Abstract excerpt

Perivascular epithelioid cell tumors (PEComas) form a family of rare mesenchymal neoplasms that typically display myomelanocytic differentiation. Upregulation of mTOR signaling due the inactivation of TSC1/2 (Tuberous Sclerosis 1 and 2) is believed to be a key oncogenic driver in this disease. Recently, a subgroup of PEComas harboring TFE3 (Transcription Factor E3) rearrangements and presenting with a distinctive...

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PMID: 33180365
DOI: 10.1002/cjp2.187
PMCID: PMC7737753

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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