ArticleModern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc2018
ZC3H7B-BCOR high-grade endometrial stromal sarcomas: a report of 17 cases of a newly defined entity.
Article in Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 54 papers.
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54 citing papers in PubMed, 169 citations in OpenAlex.
- Extraosseous Ewing sarcoma of the distal pancreas: diagnostic pitfalls and the critical role of molecular testing.Journal of surgical case reports · 2026Article
- Uterine leiomyoma-like inflammatory myofibroblastic tumor: two case reports and literature review.Frontiers in oncology · 2026Article
- High-grade uterine endometrial stromal sarcoma harboring GLI1 and MDM2/CDK4 co-amplifications.Diagnostic pathology · 2025Article
- ZC3H7A/B::BCOR fusion fibromyxoid sarcoma of soft tissue: an emerging aggressive sarcoma overlapping with malignant ossifying fibromyxoid tumors.Virchows Archiv : an international journal of pathology · 2025Article
- High-grade endometrial stromal sarcoma presenting with multiple brain metastases: a case report.Journal of medical case reports · 2025Article
- BCOR abnormalities in endometrial stromal sarcoma.Gynecologic oncology reports · 2025Review
- Integrated Transcriptomic Landscape and Deep Learning Based Survival Prediction in Uterine Sarcomas.Cancer research and treatment · 2025Article
- Immunotherapy for high-grade endometrial stromal sarcoma: a narrative review bridging molecular insights to clinical translation.Frontiers in immunology · 2025Review
- Radical tumor resection of a relapsed high-grade endometrial stromal sarcoma with an extremely rare mutation: a case report.AME case reports · 2025Article
- Uterine Mesenchymal Tumors: Updates on Pathology, Molecular Landscape, and Therapeutics.Medicina (Kaunas, Lithuania) · 2024Review
- High-grade endometrial stromal sarcoma withInternational journal of clinical and experimental pathology · 2024Article
- Prognostic factors in undifferentiated uterine sarcoma: a subanalysis of the SARCUT study.Archives of gynecology and obstetrics · 2023Article
- High-grade neuroepithelial tumor with EP300::BCOR fusion and negative BCOR immunohistochemical expression: a case report.Brain tumor pathology · 2023Article
- Breast metastasis in high-grade endometrial stromal sarcoma (HG-ESS) with BCOR rearrangement: a case report of a novel metastatic site.Diagnostic pathology · 2023Article
- Expanding the molecular spectrum of gene fusions in endometrial stromal sarcoma: Novel subunits of the chromatin remodeling complexes PRC2 and NuA4/TIP60 as alternative fusion partners.Genes, chromosomes & cancer · 2023Article
- Uterine sarcomas: clinical practice guidelines for diagnosis, treatment, and follow-up, by Spanish group for research on sarcomas (GEIS).Therapeutic advances in medical oncology · 2023Review
- Ewing and Ewing-like sarcomas: A morphological guide through genetically-defined entities.Pathology international · 2023Review
- TAZ/YAP fusion proteins: mechanistic insights and therapeutic opportunities.Trends in cancer · 2022Review
- Complex Elucidation of Cells-of-Origin in Pediatric Soft Tissue Sarcoma: From Concepts to Real Life, Hide-and-Seek through Epigenetic and Transcriptional Reprogramming.International journal of molecular sciences · 2022Review
- Desmoplastic Small Round Cell Tumor of the Uterus: A Report of Molecularly Confirmed Case with EWSR1-WT1 Fusion.Diagnostics (Basel, Switzerland) · 2022Article
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Authors and funding
22 authors at 5 institutions in 3 countries.
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Abstract
High-grade endometrial stromal sarcoma likely encompasses underrecognized tumors harboring genetic abnormalities besides YWHAE-NUTM2 fusion. Triggered by three initial endometrial stromal sarcomas with ZC3H7B-BCOR fusion characterized by high-grade morphology and aggressive clinical behavior, we herein investigate the clinicopathologic features of this genetic subset by expanding the analysis to 17 such tumors. All of them occurred in adult women with a median age of 54 (range, 28-71) years. They were predominantly based in the endomyometrium and demonstrated tongue-like and/or pushing myometrial invasion. Most were uniformly cellular and displayed haphazard fascicles of spindle cells with mild to moderate nuclear atypia. Myxoid matrix was seen in 14 of 17 (82%) tumors, and collagen plaques were seen in 8 (47%). The mitotic index was ≥10 mitotic figures/10 high-power fields (HPFs) in 14 of 17 (82%) tumors with a median of 14.5 mitotic figures/10 HPFs. No foci of conventional or variant low-grade endometrial stromal sarcoma were seen. All tumors expressed CD10 with only limited or absent desmin, SMA and/or h-caldesmon staining. ER and PR expression in >5% of cells was seen in 4 of 12 (33%) tumors. Diffuse cyclin D1 and BCOR immunoreactivity was present in 7 of 8 (88%) and 7 of 14 (50%) tumors, respectively. Fluorescence in situ hybridization or targeted RNA sequencing confirmed ZC3H7B-BCOR fusion in all tumors, including four and two previously diagnosed as myxoid leiomyosarcoma and undifferentiated uterine sarcoma, respectively. Limited clinical data suggest that patients present at higher stage and have worse prognosis compared with published outcomes in low-grade endometrial stromal sarcoma. Tumors with ZC3H7B-BCOR fusion constitute a distinct group of endometrial stromal sarcomas with high-grade morphology that should be distinguished from other uterine mesenchymal neoplasms that may demonstrate myxoid morphology.
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