Background: Sclerosing odontogenic carcinoma is a rare primary intraosseous neoplasm that was featured recently as a single entity in the World Health Organization classification of Head and Neck Tumors 2017, with only 14 cases published to date. The biological characteristics of sclerosing odontogenic carcinoma remain indistinct because of its rarity; however, it appears to be locally aggressive, with no regional or distant metastasis reported to date.
Case summary: We reported a case of sclerosing odontogenic carcinoma of the maxilla in a 62-year-old woman, who presented with an indolent right palatal swelling, which progressively increased in size over 7 years. Right subtotal maxillectomy with surgical margins of approximately 1.5 cm was performed. The patient remained disease free for 4 years following the ablation surgery. Diagnostic workups, treatment, and therapeutic outcomes were discussed.
Conclusion: More cases are needed to further characterize this entity, understand its biological behavior, and justify the treatment protocols. Resection with wide margins of approximately 1.0 to 1.5 cm is proposed, while neck dissection, post-operative radiotherapy, or chemotherapy are deemed unnecessary.
Keywords: Case report; Head and neck neoplasms; Odontogenic tumor; Sclerosing odontogenic carcinoma.
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