ArticleHead and neck pathology2025
Odontogenic Sarcomas: Clinicopathologic Analysis and Immunohistochemical Expression of BRAF and SOX9 in a Multicenter Series of 10 Cases.
Article in Head and neck pathology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers, 1 of them a synthesis that pooled it.
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Who cites it
2 citing papers in PubMed, 1 synthesis or guideline pooled it.
- BRAF p.V600E Mutation in Mixed Odontogenic Tumors and Its Clinical Correlation: A Systematic Review and Meta-Analysis.International dental journal · 2026Pooled it
- Integrating genotype-phenotype correlations into the diagnostic workflow of odontogenic tumors and cysts: insights from a 69 lesion cohort.Clinical oral investigations · 2025Article
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17 authors.
Funding
Abstract
introductionOdontogenic sarcomas (OS) are rare malignant mixed odontogenic tumors featuring benign epithelial and malignant ectomesenchymal components. MATERIALS AND
methodsTen OS cases from seven Oral and Maxillofacial Pathology services were retrospectively reviewed (2000-2024), including clinical, radiographic, histopathologic, and immunohistochemical data (BRAF p.V600E and SOX9).
resultsFive female and five male patients, with a mean age of 30 years (range: 9-72 years), presented with mandibular tumors, all as aggressive, ill-defined radiolucencies. Eight patients reported a prior diagnosis of a benign mixed odontogenic tumor (mean interval: 3.6 years). Six patients underwent wide surgical resection; one experienced recurrence within two years and showed high-grade transformation. Histopathologic examination revealed benign odontogenic epithelium within malignant ectomesenchyme with hypercellularity, pleomorphism, and increased mitotic figures. Rare features included ghost cells, microcystic degeneration, vacuolated morphology, and high-grade transformation. BRAF p.V600E was positive in 60% of the mesenchymal component. SOX9 was diffusely expressed in both epithelial and mesenchymal components, with reduced staining in the case with high-grade transformation.
conclusionOS primarily affects young adults and often arises from pre-existing benign mixed odontogenic tumors. Despite variable histology, the biological behavior of the tumors remains consistently aggressive. BRAF p.V600E and SOX9 may aid diagnosis. Vigilant follow-up of patients diagnosed with benign mixed odontogenic tumors is essential, particularly within four years post-surgery. Multicenter studies are warranted to better understand the pathogenesis of OS and support the development of targeted therapeutic strategies.
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