Background: Ameloblastic fibro-odontosarcoma (AFOS) is a rare malignant odontogenic tumor with benign epithelial and malignant mesenchymal components. It typically affects younger individuals, with a predilection for the posterior mandible. With fewer than 25 cases reported globally, it remains a diagnostic challenge due to overlapping features with other odontogenic lesions and limited documented information.
Case presentation: This is a case of a 21-year-old male who presented with a progressively enlarging swelling on the right side of his face, painful growth in the posterior mandible, and numbness in the right lower lip. Clinical examination revealed a bony hard swelling with an ulcerative lesion in the mandibular alveolar mucosa. Cone-beam computed tomography showed a heterogeneously hypodense, expansile lesion with bicortical expansion, cortical erosion, and hyperdense masses resembling enamel and dentin. Histopathological analysis confirmed AFOS, showing benign odontogenic epithelial islands within a malignant mesenchymal stroma. The patient underwent surgical resection with wide margins, followed by radiotherapy and chemotherapy to address metastatic lymph node involvement.
Conclusion: This case highlights the diagnostic complexities and aggressive behavior of AFOS. A multidisciplinary approach integrating clinical, radiographic, and histopathological findings is critical for accurate diagnosis and effective management.