ArticleVirchows Archiv : an international journal of pathology2023
"PRRX1-rearranged mesenchymal tumors": expanding the immunohistochemical profile and molecular spectrum of a recently described entity with the proposed revision of nomenclature.
Article in Virchows Archiv : an international journal of pathology, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers.
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Who cites it
9 citing papers in PubMed, 14 citations in OpenAlex.
- Review
- ACTIN::MITF-rearranged tumor: a distinctive entity or a cutaneous PEComa with MITF alterations? A clinicopathological and molecular study of additional 5 cases.Virchows Archiv : an international journal of pathology · 2026Article
- Expanding the Anatomical Distribution of PRRX1::KMT2D Fusion Mesenchymal Neoplasms: A Rare Mediastinal Case Report.Cancer reports (Hoboken, N.J.) · 2026Article
- FET-Rearranged Myoepithelial Tumors Are Clinically Heterogeneous and Epigenetically Distinct from PLAG1-Rearranged Adnexal and Salivary Gland Myoepithelial Tumors.Clinical cancer research : an official journal of the American Association for Cancer Research · 2026Article
- ESR1::NCOA2/3 fusions in uterine neoplasms with adenosarcoma-like morphology: clinicopathologic and molecular features of 12 cases and review of the literature.Virchows Archiv : an international journal of pathology · 2026Article
- PRRX1 -rearranged Fibroblastic Tumors : A Clinicopathologic and Molecular Study of 18 Cases Including a Novel PRRX1::EP300 Fusion.The American journal of surgical pathology · 2026Article
- Clinicopathologic and Molecular Genetic Features of Spindle Cell Rhabdomyosarcoma Harboring ZFP64::NCOA2/3 Fusions: A Series of 14 Cases.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2025Article
- GLI1::FOXO4-rearranged kidney tumors: a potentially distinct renal subtype within the spectrum of GLI1-altered tumors?Virchows Archiv : an international journal of pathology · 2025Article
- What is new in fibroblastic/myofibroblastic tumors in children.Virchows Archiv : an international journal of pathology · 2025Review
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Authors and funding
10 authors at 8 institutions in 5 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Since the publication of the 2020 World Health Organization classification of soft tissue and bone tumors, the classification of "fibroblastic" tumors has expanded to include a novel subset of tumors characterized by PRRX1::NCOA1/2 gene fusions. These tumors defy conventional classification and are morphologically distinct, characterized by a multi-nodular growth of bland spindle cells suspended in a myxo-collagenous stroma with mild cytologic atypia, "staghorn-like" vessels, and variable perivascular hyalinization. Mitotic activity is rare, and necrosis is not identified. Herein, we present six additional cases of PRRX1-rearranged mesenchymal tumors, including five cases with PRRX1::NCOA1 fusion and one case with PRRX1::KMT2D fusion. Three cases (3/6, 50%) demonstrated focal co-expression of S100 protein and SOX10, thereby expanding the immunohistochemical profile of this emerging entity. Like prior reported cases, there was no evidence of malignant behavior on short-term follow-up. The novel fusion, PRRX1::KMT2D, further expands the molecular spectrum of this entity and leads to a proposed revision of the provisional nomenclature to "PRRX1-rearranged mesenchymal tumor" to both accommodate non-NCOA1/2 fusion partners and allow for the possibility of partial neural or neuroectodermal differentiation.
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