Odontogenic carcinosarcoma, a rare and challenging diagnosis, was identified in a 60-year-old male through histopathology, revealing a biphasic neoplasm with malignant epithelial and mesenchymal components. Surgical resection is crucial for management, highlighting the importance of vigilant postoperative follow-up to ensure early detection of any recurrence. One rare mixed malignant odontogenic tumor is odontogenic carcinosarcoma, which comprises malignant epithelial and mesenchymal components. Diagnosing odontogenic carcinosarcoma is challenging due to its rarity and atypical clinical presentation. This study reports a 60-year-old male patient who presented with a painless swelling on the right side of his face and experienced facial asymmetry for 6 months, ultimately diagnosed with odontogenic carcinosarcoma. A biphasic neoplasm with malignant alterations in both epithelial and mesenchymal components was identified upon histopathological examination. MRI imaging showed an expansile multilobulated lytic lesion with cortical erosion and extraosseous extension in the posterior region of the right mandibular body and ramus. Following contrast administration, homogeneous lesion enhancement was observed, with a few small non-enhancing necrotic areas in central parts. The patient subsequently underwent a right hemi-mandibulectomy with resection of adjacent soft tissues and neck dissection due to lymph node involvement. The resulting defect was reconstructed using a pectoralis major flap. No recurrence or metastasis was reported during the 6-month follow-up, reinforcing the positive results. This case highlights the importance of recognizing odontogenic carcinosarcoma and underscores the challenges in diagnosing and managing this rare tumor. Early identification and aggressive treatment can lead to positive outcomes, as evidenced by the absence of recurrence or metastasis in this patient during the follow-up period.
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