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Introduction <p>Odontogenic sarcomas (OS) are rare malignant mixed odontogenic tumors featuring benign epithelial and malignant ectomesenchymal components.</p> Materials and methods <p>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).</p> Results <p>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.</p> Conclusion <p>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.</p>

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Odontogenic Sarcomas: Clinicopathologic Analysis and Immunohistochemical Expression of BRAF and SOX9 in a Multicenter Series of 10 Cases

  • 期刊论文

  • 发表日期：

    2025年8月8日

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  • Ana Luiza Oliveira Corrêa Roza,
  • Stephanie Vargas de Freitas,
  • Chané Smit,
  • André Caroli Rocha,
  • Michelle Agostini,
  • Ellen Brilhante Cortezzi,
  • Aline Corrêa Abrahão,
  • Brian Shumway,
  • Madhu Shrestha,
  • Letícia Ferreira Cabido,
  • Victoria Woo,
  • Benjamin Martínez-Rondanelli,
  • Adalberto Mosqueda-Taylor,
  • Willie F. P. van Heerden,
  • John M. Wright,
  • Pablo Agustin Vargas,
  • Mário José Romañach

摘要

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.