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Odontogenic Sarcomas: Clinicopathologic Analysis and Immunohistochemical Expression of BRAF and SOX9 in a Multicenter
Ana Luiza Oliveira Corrêa Roza1, Stephanie Vargas de Freitas2, Chané Smit3
1Department of Oral Diagnosis, Piracicaba Dental School, University of Campinas (UNICAMP), Piracicaba, Brazil.
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.
