Abstract
INTRODUCTION: Adenoid ameloblastoma (AdAM) is a rare epithelial odontogenic tumour recently included in the 5th World Health Organization classification of head and neck tumours as a separate tumour from conventional ameloblastoma. It presents diagnostic and management challenges due to its morphological overlapping features with adenomatoid odontogenic tumour (AOT) and conventional ameloblastoma. CASE REPORT: A 32-year-old female with neurofibromatosis type I presented with a 4-month history of right-sided facial swelling. Clinical examination revealed a firm, expansive mass in the right anterior mandibular region with associated tooth mobility. Radiographic evaluation demonstrated a well-demarcated unilocular radiolucency mimicking a cystic lesion. Histological examination revealed an epithelial odontogenic neoplasm composted of nests of ovoid to spindle-shaped cells demonstrating pseudo-duct-like spaces, cribriform areas and a whirling pattern with deposition of dentinoid material. These features confirmed the diagnosis of AdAM. The tumour was resected and immediately reconstructed with a fibula free flap. The patient remains diseases-free 2 years post-surgery. CONCLUSION: There are some overlapping clinico-pathological features of this tumour with AOT, which has led to inadequate surgical management previously with high recurrence rates. Emphasis should be placed on identifying all histopathological features described to arrive at an accurate diagnosis. Surgical treatment of AdAM should be radical resection despite its clinical and radiographic presentation mimicking the less aggressive AOT.