Keratoameloblastoma: Report of a rare variant with review of literature

Ameloblastoma has intrigued clinicians as well as pathologists due to its diverse clinical behavior and histomorphologic presentations. Keratoameloblastoma is a rare histologic sub type, characterized by extensive keratin formation within ameloblastic epithelium, with only a handful number of cases described in the literature. Here, we report a case of this uncommon sub type of ameloblastoma in a young female patient presenting as an extensive lesion in mandibular ramus area. The radiological and fine needle aspiration findings suggested of a keratinizing cystic lesion and incisional biopsy showed features of ameloblastoma. Patient underwent segmental mandibulectomy and histological examination of excisional specimen revealed features of ameloblastoma with abundant keratinization leading to a diagnosis of keratoameloblastoma. The diagnostic pitfalls related with the lesion have been discussed along with a short review of the literature.