ArticleThe American journal of surgical pathology2019
Spindle and Round Cell Sarcoma With EWSR1-PATZ1 Gene Fusion: A Sarcoma With Polyphenotypic Differentiation.
Article in The American journal of surgical pathology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 33 papers.
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Who cites it
33 citing papers in PubMed, 75 citations in OpenAlex.
- Unveiling a genetic rarity: intracranial sarcomatous tumor with EWSR1::PATZ1 fusion-a case report and review of the literature.Brain tumor pathology · 2026Article
- Clinicopathologic correlates of Ewing (FET::ETS-rearranged) sarcoma and non-Ewing undifferentiated small round cell sarcomas across skeletal and extraskeletal sites.Frontiers in oncology · 2026Article
- Indolent Abdominal Wall Round Cell Sarcoma With EWSR1-PATZ1 Fusion: A Case Report and Literature Review.Cureus · 2025Article
- Emerging round cell sarcomas in children.Virchows Archiv : an international journal of pathology · 2025Review
- WHO is embedding inequality into cancer diagnostics.BMJ oncology · 2025Article
- Fusion-driven cutaneous and superficial mesenchymal and adnexal tumors-A clinicopathologic and molecular study of 15 cases, including a novel case of ACTB::ZMIZ2-rearranged adnexal carcinoma.Journal of cutaneous pathology · 2024Article
- A case of pelvicRadiology case reports · 2024Article
- Primary Ewing's sarcoma of the uterine cervix: a case report and review of the literature.Journal of cancer research and clinical oncology · 2024Review
- Sarcomas with EWSR1::Non-ETS Fusion (EWSR1::NFATC2 and EWSR1::PATZ1).Surgical pathology clinics · 2024Review
- Rare posterior fossa EWSR1-PATZ1 gene fusion glioneuronal tumour-mimicking ependymoma in an adolescent successfully treated with surgery alone.BMJ case reports · 2023Article
- The Recent Advances in Molecular Diagnosis of Soft Tissue Tumors.International journal of molecular sciences · 2023Review
- Top 10 Histological Mimics of Neuroendocrine Carcinoma You Should Not Miss in the Head and Neck.Head and neck pathology · 2023Review
- Ewing and Ewing-like sarcomas: A morphological guide through genetically-defined entities.Pathology international · 2023Review
- An international working group consensus report for the prioritization of molecular biomarkers for Ewing sarcoma.NPJ precision oncology · 2022Review
- Adamantinoma-Like Ewing Sarcoma of the Head and Neck: A Case-Series of a Rare and Challenging Diagnosis.Head and neck pathology · 2022Article
- WHO Pathology: Highlights of the 2020 Sarcoma Update.Surgical oncology clinics of North America · 2022Review
- Development and validation of an RNA sequencing panel for gene fusions in soft tissue sarcoma.Cancer science · 2022Article
- A novel LARGE1-AFF2 fusion expanding the molecular alterations associated with the methylation class of neuroepithelial tumors with PATZ1 fusions.Acta neuropathologica communications · 2022Article
- Round Cell Sarcoma with EWSR1-PATZ1 Fusion in the Face of a Five-Year-Old Boy: Report of a Case with Unusual Histologic Features.Head and neck pathology · 2021Article
- PATZ1 fusions define a novel molecularly distinct neuroepithelial tumor entity with a broad histological spectrum.Acta neuropathologica · 2021Article
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Authors and funding
8 authors at 1 institution in 1 country.
Funding
Abstract
The evolving classification of round cell sarcomas is driven by molecular alterations. EWSR1-PATZ1 fusion positive spindle and round cell sarcoma is one such new tumor entity. Herein, we report 2 EWSR1-PATZ1 fusion positive spindle and round cell sarcomas with overlapping histologic features and polyphenotypic differentiation. The intra-abdominal tumors affected female patients, 31-and 53-year old. Both tumors showed sheets and nests of round to spindle cells, fine chromatin, tiny conspicuous nucleoli, moderate cytoplasm, and thick bands of intratumoral fibrosis. On immunohistochemistry, both tumors showed positivity for CD99, desmin, myogenin, MyoD1, S100, Sox10, CD34, and GFAP and were negative for keratin. Fluorescence in situ hybridization revealed rearrangement at EWSR1 locus. Next-generation sequencing-based RNA fusion assay revealed EWSR1-PATZ1 fusion in both cases. EWSR1-PATZ1 fusion positive spindle and round cell sarcomas show abundant intratumoral fibrosis and polyphenotypic differentiation, thus mimicking a range of tumors including desmoplastic small round cell tumor. The precise classification of this spindle and round cell sarcoma and its relationship to the Ewing sarcoma family of tumors remains to be determined.
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