ArticleThe American journal of surgical pathology2019
Novel PLAG1 Gene Rearrangement Distinguishes a Subset of Uterine Myxoid Leiomyosarcoma From Other Uterine Myxoid Mesenchymal Tumors.
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 35 papers.
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Who cites it
35 citing papers in PubMed, 95 citations in OpenAlex.
- Therapeutic Vulnerabilities of the Key Genetic Drivers in Leiomyosarcoma.Medical sciences (Basel, Switzerland) · 2026Review
- Multicenter Study of MRI Features in Uterine Myxoid Leiomyosarcoma: Revealing and Overcoming a Critical Pitfall in Current Consensus Criteria.Korean journal of radiology · 2026Article
- Intra-Abdominal Myxoid Leiomyosarcoma With PLAG1 Gene Rearrangement Following Prior Laparoscopic Myomectomy: A Case Report and Literature Review.Clinical case reports · 2026Article
- Two pediatric supratentorial ependymal tumors with novel PLAG1 fusions.Acta neuropathologica communications · 2026Article
- TRPS1::PLAG1 Fusion in a Primary Cutaneous Myoepithelial Carcinoma: A Case Report and Literature Review.Journal of cutaneous pathology · 2026Review
- Pre-Treatment BOC Expression as an Indicator of Lymphovascular Invasion andOncology research · 2026Article
- The Evolving Molecular Landscape of Uterine Mesenchymal Tumors: Diagnostic and Therapeutic Implications.Cancers · 2025Review
- CD34-positive pleomorphic uterine sarcoma with NUDT3::RAD51B fusion.Virchows Archiv : an international journal of pathology · 2025Article
- Leiomyosarcoma Therapeutic Approaches and Future Directions.Hematology/oncology clinics of North America · 2025Review
- Uterine Mesenchymal Neoplasm With a Novel CDC42::PLAG1 Fusion.Pathology international · 2025Article
- Comprehensive pan-cancer analysis identifies PLAG1 as a key regulator of tumor immune microenvironment and prognostic biomarker.Frontiers in immunology · 2025Article
- Fibromyxoid aSoft Tissue Tumor With PLAG1 Fusion-The First Case in an Adult Patient.Genes, chromosomes & cancer · 2024Article
- ERBB2/ ERBB3-mutated S100/ SOX10-positive unclassified high-grade uterine sarcoma: first detailed description of a novel entity.Virchows Archiv : an international journal of pathology · 2024Article
- Pediatric fibromyxoid brachial plexus tumor with YWHAZ::PLAG1 gene fusion: a case report.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2024Article
- Sarcomas With RAD51B Fusions Are Associated With a Heterogeneous Phenotype.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2024Article
- High-grade endometrial stromal sarcoma withInternational journal of clinical and experimental pathology · 2024Article
- A dysfunctional miR-1-TRPS1-MYOG axis drives ERMS by suppressing terminal myogenic differentiation.Molecular therapy : the journal of the American Society of Gene Therapy · 2023Article
- Modeling sarcoma relevant translocations using CRISPR-Cas9 in human embryonic stem derived mesenchymal precursors.Genes, chromosomes & cancer · 2023Review
- An Extremely Rare Case of Disseminated Peritoneal Leiomyomatosis with a Pelvic Leiomyosarcoma and Omental Metastasis after Laparoscopic Morcellation: Systematic Review of the Literature.Diagnostics (Basel, Switzerland) · 2022Article
- Expanding the clinicopathological spectrum of TGFBR3-PLAG1 rearranged salivary gland neoplasms with myoepithelial differentiation including evidence of high-grade transformation.Genes, chromosomes & cancer · 2022Article
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12 authors at 5 institutions in 2 countries.
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Abstract
Genetic alterations in uterine myxoid leiomyosarcoma are unknown. We investigate the clinicopathologic features of 19 uterine tumors previously diagnosed as myxoid leiomyosarcomas in which tumoral RNA was subjected to targeted RNA sequencing. PLAG1, BCOR, BCORL1, HMGA2, and ALK break-apart fluorescence in situ hybridization (FISH) and BCOR, PLAG1, and ALK immunohistochemistry were performed in cases which failed or lacked fusions by sequencing. The diagnosis of myxoid leiomyosarcoma was confirmed in 15 cases after exclusion of 4 tumors with BCOR and ALK rearrangements. These 15 patients presented at a median age of 50 years with stage I (3), II (2), III (2), and IV (1) tumors, respectively; stage was unknown in 7 cases. Tumor size ranged from 10 to 24 cm. Matrix was myxoid in all tumors and also eosinophilic in 2. Cells were spindled, epithelioid, and both in 10, 2, and 3 tumors and showed mild, moderate, and severe nuclear atypia in 3, 8, and 4 tumors, respectively. Mitotic index ranged from <1 to 14/10 HPF, while tumor necrosis was present in 6 (40%). Novel TRPS1-PLAG1 or RAD51B-PLAG1 fusions were detected by sequencing in 4 tumors, 3 of which were also confirmed by FISH. Diffuse PLAG1 expression was seen in 7 tumors, including 4 with PLAG1 rearrangement. No morphologic differences were seen among PLAG1 fusion-positive and fusion-negative tumors. No PLAG1, HMGA2, ALK, BCOR, or BCORL1 rearrangements were detected by FISH in 11 tumors. On the basis of sequencing and FISH results, PLAG1 rearrangements resulting in PLAG1 expression underpin ~25% of myxoid leiomyosarcomas and may serve as a useful diagnostic biomarker. Immunohistochemistry, targeted RNA sequencing, and/or FISH may distinguish myxoid leiomyosarcoma from its morphologic mimics.
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