USP8-Rearranged Mesenchymal Tumors With Myofibroblastic Phenotype: A Comprehensive Clinicopathologic, Genetic, and Epigenetic Characterization.

doi:10.1016/j.modpat.2025.100947

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Article

USP8-Rearranged Mesenchymal Tumors With Myofibroblastic Phenotype: A Comprehensive Clinicopathologic, Genetic, and Epigenetic Characterization.

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

Arciuolo Damiano, Barresi Sabina, Hiemcke-Jiwa Laura, Black Jennifer, Willard Nicholas, Carta Roberto, Roe Michelle, Bukowinski Andrew, Stracuzzi Alessandra, Kester Lennart, Koudijs Marco, Dingemans Willemijn, Milano Giuseppe Maria, Patrizi Sara, Gestrich Catherine, John Ivy, Azfar Neyaz, Bubar Robert, Skaugen John, Flucke Uta, Miele Evelina, Alaggio Rita

Abstract excerpt

USP8 is one of the members of ubiquitin-specific proteases deconjugating ubiquitin from target proteins. Besides USP6, it can be involved in tumorigenesis of mesenchymal neoplasms by binding to an activating fusion partner. Until now, USP8 fusion genes have been reported in calcified chondroid mesenchymal neoplasms, an inflammatory myofibroblastic tumor, a cardiac neoplasm, and a retroperitoneal sarcoma. In this...

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Identifiers

PMID: 41380847
DOI: 10.1016/j.modpat.2025.100947

Genes

USP8
USP6
CRK
FARP1
PDGFRA
PTPN11
RASA1
SH3KBP1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 4 Sept 2026.

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