Keratoameloblastoma: unusual keratinization in ameloblastoma: a case report and review of literature.

OBJECTIVE

To present a case of keratoameloblastoma (KA), a rare keratinizing ameloblastoma variant, and review its diagnostic and management features.

STUDY

DESIGN

A 69-year-old man with a mandibular radiolucent lesion underwent clinical, radiographic, and histopathologic evaluation, and a literature review was conducted.

RESULTS

Imaging showed a large unilocular radiolucency extending from the mandibular ramus to the distal aspect of tooth #30. Histology revealed epithelial follicles with extensive lamellated parakeratin, keratin pearl formation, focal stellate reticulum-like areas, and palisaded columnar cells with reverse polarity. Tumor cells were positive for AE1/AE3, p63, and nuclear β-catenin, with a low Ki-67 index (<2%); BRAF V600E was negative. The patient underwent mandibular resection with clear margins.

CONCLUSION(S): KA is a rare ameloblastoma variant that can mimic other keratinizing odontogenic lesions. Despite its distinctive histology, it behaves similarly to conventional ameloblastoma, underscoring the importance of accurate diagnosis and adequate surgical excision.