Roza, Ana Luiza Oliveira CorreaDe Freitas, Stephanie VargasSmit, ChaneRocha, Andre CaroliAgostini, MichelleCortezzi, Ellen BrilhanteAbrahao, Aline CorreaShumway, BrianShrestha, MadhuCabido, Leticia FerreiraWoo, VictoriaMartinez-Rondanelli, BenjaminMosqueda-Taylor, AdalbertoVan Heerden, Willem Francois PetrusWright, John M.Vargas, Pablo AgustinRomanach, Mario Jose2025-10-072025-08Roza, A.L.O.C., de Freitas, S.V., Smit, C. et al. Odontogenic Sarcomas: Clinicopathologic Analysis and Immunohistochemical Expression of BRAF and SOX9 in a Multicenter Series of 10 Cases. Head and Neck Pathology 19, 99 (2025). https://doi.org/10.1007/s12105-025-01817-8.1936-055X (print)1936-0568 (online)10.1007/s12105-025-01817-8http://hdl.handle.net/2263/104631DATA AVAILABILITY : No datasets were generated or analysed during the current study.INTRODUCTION : Odontogenic sarcomas (OS) are rare malignant mixed odontogenic tumors featuring benign epithelial and malignant ectomesenchymal components. MATERIALS AND METHODS : Ten 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). RESULTS : Five 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. CONCLUSION : OS 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.en© The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature 2025. The original publication is available at : https://link.springer.com/journal/12105.Odontogenic sarcomasAmeloblastic fibrosarcomaAmeloblastic fibrodentinosarcomaAmeloblastic fibro-odontosarcomaBRAF p.V600ESOX9Postprint Article