Abstract
Background
Molecular diagnostics has greatly refined sinonasal tumor pathology over the past decade. While much of the attention has focused on carcinomas, it is becoming clear that there are emerging mesenchymal neoplasms which have previously defied classification.
Methods
Here, we present a 33-year-old woman with a multiply recurrent sinonasal spindle cell tumor exhibiting distinctive features, and not easily classifiable into a specific category.
Results
The hypercellular tumor was composed of plump spindled cells, with uniform vesicular chromatin arranged as vague fascicles around a prominent hemangiopericytoma-like vasculature. The mitotic rate was brisk at 10 per 10 high power fields. By immunohistochemistry, it was only positive for EMA (focal) and SATB2 (diffuse, weak). Fusion analysis uncovered EWSR1::BEND2, a fusion which is best known for being seen in astroblastoma, but which has not yet been reported in sarcomas.
Conclusion
This case underscores the utility of fusion analysis when confronted with a sinonasal spindle cell neoplasm which does not neatly fit into any specific category. It remains to be seen if EWSR1::BEND2 sinonasal sarcoma represents a distinct entity.
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Data Availability
All data generated or analyzed during this study are included in this published article.
Code Availability
Not applicable.
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Funding
This study was funded by the Jane B. and Edwin P. Jenevein M.D Endowment for Pathology at UT Southwestern Medical Center. No external funding was obtained for this study.
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VM and JAB designed the study, performed data collection and interpretation, and prepared the manuscript. JYP performed data collection and interpretation. All authors read and approved the final paper.
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Palsgrove, D.N., Manucha, V., Park, J.Y. et al. A Low-grade Sinonasal Sarcoma Harboring EWSR1::BEND2: Expanding the Differential Diagnosis of Sinonasal Spindle Cell Neoplasms. Head and Neck Pathol 17, 571–575 (2023). https://doi.org/10.1007/s12105-023-01527-z
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DOI: https://doi.org/10.1007/s12105-023-01527-z