ArticleVirchows Archiv : an international journal of pathology2023
Biphenotypic sinonasal sarcoma with PAX3::MAML3 fusion transforming into high-grade rhabdomyosarcoma: report of an emerging rare phenomenon.
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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9 citing papers in PubMed, 16 citations in OpenAlex.
- Fusion-positive rhabdomyosarcoma oncofusions share a common interactome.Nature communications · 2026Article
- A Genomic and Epigenetic Comparative Study of Low-grade Biphenotypic Sinonasal Sarcoma with Metachronous and Synchronous High-grade Rhabdomyosarcomatous Transformation.Head and neck pathology · 2026Article
- Biphenotypic Sinonasal Sarcoma: Literature Review of a Peculiar Pathological Entity-The Neurosurgical Point of View.Cancers · 2024Review
- Biphenotypic Sinonasal Sarcoma with Orbital Invasion: A Literature Review and Modular System of Surgical Approaches.Cancers · 2024Review
- Molecularly defined sinonasal malignancies: an overview with focus on the current WHO classification and recently described provisional entities.Virchows Archiv : an international journal of pathology · 2024Review
- Spindle Cell Tumors of the Sinonasal Tract: A Diagnostic Update with Focus on Ancillary Workup.Head and neck pathology · 2024Review
- Molecular pathology in diagnosis and prognostication of head and neck tumors.Virchows Archiv : an international journal of pathology · 2024Review
- Biphenotypic Sinonasal Sarcoma with a Novel PAX7::PPARGC1 Fusion: Expanding the Spectrum of Gene Fusions Beyond the PAX3 Gene.Head and neck pathology · 2023Article
- [Special mesenchymal neoplasms of the head and neck: Update from the 2022 WHO].Pathologie (Heidelberg, Germany) · 2023Review
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7 authors at 4 institutions in 2 countries.
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Abstract
We report a case of a 67-year-old male patient with a sinonasal tumor that showed areas of classic biphenotypic sinonasal sarcoma (BSNS) which in some sections sharply transitioned into high-grade rhabdomyosarcoma. Immunohistochemically, the conventional BSNS parts showed S100 protein, SMA, PAX7, and focal MyoD1 expression, whereas desmin and myogenin were negative. In contrast, the cells in high-grade areas expressed desmin, MyoD1, myogenin, and PAX7, while being negative for S100 protein and SMA. Using the Archer FusionPlex assay, the classical PAX3::MAML3 gene fusion was detected. FISH for PAX3 and MAML3 confirmed a break of these genes in both components. Despite aggressive therapy, the tumor progression resulted in the patient's death. The herein presented case, together with 2 previously published cases of BSNS with high-grade transformation, helps to better understand this novel phenomenon. Although the risk for such transformation appears low, it has important clinical and diagnostic implications which are discussed.
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