Primary Mandibular Odontogenic Clear Cell Carcinoma: A Case Report

Odontogenic clear cell carcinoma (CCC) of the mandible is a rare malignancy with limited literature. This case underscores the diagnostic challenges and emphasizes multidisciplinary strategies for managing this entity, highlighting its relevance in oral oncology. A 45-year-old male presented with an asymptomatic left mandibular mass discovered incidentally during dental imaging. Physical examination revealed no lymphadenopathy or facial asymmetry. Biopsy confirmed low-grade odontogenic CCC characterized by clear cytoplasmic tumor cells and epithelial islands. PET-CT localized the lesion at the anterior border of left ramus without metastasis, secondary findings only included benign renal cysts. No malignant primary renal tumors were observed. Preoperative evaluation excluded surgical contraindications. The surgery was performed with a 3D-printed mandibular model and surgical template. Extended resection of the tumor and segmental mandibulectomy were conducted, a fibula free flap was used for subsequent reconstruction. The rarity and histologically overlapping characteristics of mandibular CCC with other malignant and benign tumors necessitate rigorous pathology and imaging like PET-CT. The absence of lymphadenopathy aligns with low-grade behavior, although delayed metastasis remains a concern. Incidental renal cysts highlight the importance of systemic evaluation in patients with cancer. The restoration of masticatory function and maxillofacial appearance was verified to be successful. Early histopathological confirmation and multidisciplinary collaboration are essential. Despite its low-grade features, long-term surveillance is critical for the detection of recurrence. This case reinforces the need for integrated care for rare malignancies, balancing oncological rigor with holistic patient management.