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REVIEW article
Front. Oral. Health
Sec. Oral Cancers
Volume 6 - 2025 | doi: 10.3389/froh.2025.1544921
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Malignant odontogenic tumors are rare, accounting for only 1-6.1% of all odontogenic tumors. Among them, odontogenic carcinosarcoma (OCS) is an exceptionally rare and aggressive malignant neoplasm originating from dental tissues. First recognized by the World Health Organization (WHO) in 1992, OCS is characterized by high-grade biphasic malignant epithelial and mesenchymal components, contributing to its aggressive clinical behavior. OCS often presents with nonspecific symptoms such as pain, swelling, and loosening of teeth, which complicate early diagnosis. Its rarity adds to the diagnostic challenges, frequently leading to delays in identification. Histopathological evaluation remains the cornerstone for accurate diagnosis, distinguishing OCS from other odontogenic tumors through features like epithelial nuclear pleomorphism, mitotic activity, and mesenchymal sarcomatous differentiation. Management typically involves surgical resection with clear margins, while adjuvant therapies such as chemotherapy and radiation are considered in select cases. Recent advancements in molecular oncology and surgical techniques, including robotic-assisted procedures and 3Dprinted reconstructive aids, offer promising avenues for improving patient outcomes. A multidisciplinary approach and ongoing research are essential to enhance diagnostic accuracy, refine treatment protocols, and improve the prognosis for patients affected by this rare malignancy. The primary objective of this review is to consolidate current knowledge on OCS, focusing on its diagnostic complexities, treatment strategies, and potential emerging therapies.
Keywords: Odontogenic Tumors, Mouth Neoplasms, immune checkpoint inhibitors, Molecular Targeted Therapy, OCS
Received: 13 Dec 2024; Accepted: 26 Mar 2025.
Copyright: © 2025 Osama, Kocherry, Ullah, Ubaid, Ubaid, Ullah, Nawaz, Qasem, Odat, Farhan and Ahmed. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence:
Raheel Ahmed, Imperial College London, London, SW7 2AZ, England, United Kingdom
Disclaimer: All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
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