Orbital Ameloblastic Carcinoma – Unusual Presentation in A Non-Odontogenic Location

 

Abstract

Ameloblastic carcinoma has been described as an ameloblastoma in which there is histological evidence of carcinoma in a primary or recurrent ameloblastoma. The frequency of the lesion is estimated to be less than 1% of all ameloblastomas occurring in the mandible and maxilla.1 We present a case of a 30-year-old woman who presented with painless swelling of left upper face and recurrent upper respiratory tract infections. She was evaluated with CECT, MRI and PET-CT which showed 3.6 x 3.5 x 3.3 cm mass arising from the inferomedial wall of left orbit with sunburst periosteal reaction and cloudy osteoid matrix which was thought to be an osteosarcoma. She underwent a transorbital and trans nasal endoscopic wide local excision of the lesion which was then diagnosed as ameloblastic carcinoma on histopathology. Imaging features and pathological findings of the tumor along with novel treatment strategies and differential diagnosis are discussed in this case report.

Keywords: MRI; orbital tumor; ameloblastic carcinoma; osteosarcoma; CECT; PET

Abbreviations: 18F-FDG-PET: 18F-fluorodeoxyglucose–Positron Emission Tomography; CECT: Contrast Enhanced Computed Tomography; FLAIR: Fluid Attenuated Inversion Recovery; MRI: Magnetic Resonance Imaging; WHO: World Health Organization

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