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Article

Alternative Lengthening of Telomeres in Malignant Perivascular Epithelioid Cell Neoplasms: Correlation With Molecular Features Including ATRX Gene Mutation Status.

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc - 1 Mar 2026

Lin Ruihe, Gross John M, Xing Deyin, Argani Pedram, Lotan Tamara, Meeker Alan K, Baraban Ezra G

Abstract excerpt

A subset of perivascular epithelioid cell neoplasms (PEComas) is histologically malignant and at high risk for metastasis, and there is limited literature available on the genetic features of these lesions. In addition to driver alterations in the tuberous sclerosis complex/mammalian target of rapamycin pathway or TFE3 fusions, recurrent ATRX mutations have been identified in malignant PEComas. ATRX mutations...

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Identifiers

PMID: 41577032
DOI: 10.1016/j.modpat.2026.100964

Genes

ATRX
RICTOR
TSC1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026.

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Alternative Lengthening of Telomeres in Malignant Perivascular Epithelioid Cell Neoplasms: Correlation With Molecular Features Including ATRX Gene Mutation Status.PMID 41577032Read ↗

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