Keratoameloblastoma: Unusual Keratinization in Ameloblastoma – A Case Report and Review of Literature

Objective(s)To present a case of keratoameloblastoma (KA), a rare keratinizing variant of ameloblastoma and review its clinical, radiologic, immunohistochemical, and molecular features, highlighting diagnostic challenges and management. Study DesignA 69-year-old man with a mandibular radiolucent lesion underwent clinical, radiographic, and histopathologic evaluation and was diagnosed with keratoameloblastoma.Additionally, a literature review was conducted, summarizing clinical, radiologic, histopathologic, immunophenotypic, and molecular features as well as treatment modalities for this rare entity. ResultsImaging demonstrated a large unilocular radiolucency extending from the mandibular ramus to the distal aspect of tooth #30.Histologic evaluation revealed epithelial follicles with prominent lamellated parakeratin, keratin pearl formation, and scattered stellate-reticulum-like areas, bordered by palisaded columnar cells with reverse polarity.The immunoprofile showed positivity for AE1/AE3, p63, and nuclear -catenin with a low Ki-67 index (<2%).BRAF V600E expression was negative.The patient underwent mandibular resection with clear margins. Conclusion(s)Keratoameloblastoma is an uncommon ameloblastoma variant with extensive keratinization that may mimic other odontogenic lesions.Although morphologically distinctive, KA exhibits similar clinical behavior to conventional ameloblastoma, with potential for local aggressiveness and recurrence if inadequately excised.Recognition of its histopathologic spectrum is essential to avoid misdiagnosis and ensure appropriate surgical management.