Odontogenic Sarcoma of the Mandible: A Rare Case That Poses Significant Challenges to Diagnosis

Abstract

Odontogenic sarcoma is a rare biphasic odontogenic malignancy composed of benign odontoid epithelium and malignant mesenchymal stroma. A review of the literature revealed approximately 100 documented cases. The clinical and histopathological heterogeneity of the tumor can lead to diagnostic challenges, particularly in cases where limited biopsies are conducted. A 22-year-old female patient was evaluated for a rapidly growing mass in the posterior region of the mandible. Computerized tomography imaging revealed a mass originating from the right mandible. Because no specific line of differentiation was identified on histopathological examination and the immunohistochemical markers were nonspecific, the differential diagnosis included Ewing sarcoma, synovial sarcoma, spindle cell carcinoma, and small round cell tumors and mesenchymal neoplasms. It was ascertained that the patient had previously undergone a biopsy, the results of which were not available, and the old specimens were requested. Subsequent re-evaluation of the original specimens revealed the presence of an odontogenic epithelial component, thus leading to the diagnosis of odontogenic sarcoma. Odontogenic sarcoma can lead to diagnostic errors if the epithelial component cannot be detected in small biopsies.