Abstract
Odontogenic carcinosarcoma is a very rare malignant odontogenic tumor, characterized by malignant epithelial and mesenchymal components. Studies have reported several cases of odontogenic carcinosarcoma, mainly in the upper and lower jaws, with malignant clinical manifestations. Herein, we present the case of a 58-year-old woman with odontogenic carcinosarcoma with dentinoid in the left maxilla. The invasion range was large, and the left maxillary molar was missing. Histology revealed odontogenic carcinosarcoma with bidirectional differentiation characteristics and comprising three components: malignant epithelium, malignant interstitium, and dentinoid. The patient subsequently underwent nasal endoscopic sinus tumor resection, and she recovered well after surgery. After a strict 4-year follow-up, to date, there are still no signs of disease or local recurrence. To our knowledge, this is the first reported case of odontogenic carcinosarcoma with dentinoid. Our study describes the clinical, morphological, and immunohistochemical characteristics of this case, and distinguishes it from related diseases.