Ameloblastoma: An Updated Narrative Review of an Enigmatic Tumor

Abstract

Ameloblastoma is one of the most common benign odontogenic tumors of the jaw that constitutes about 10% of all tumors that arise in the mandible and maxilla. It is a slow-growing but locally invasive tumor that presents with painless swelling of the mandible or maxilla. The World Health Organization (WHO) classification of 2017 describes ameloblastomas of the following four types: ameloblastoma; unicystic ameloblastoma; extraosseous/peripheral ameloblastoma; and metastasizing ameloblastoma. The diagnosis of ameloblastoma requires computerized tomography (CT) imaging as well as a biopsy. A biopsy is helpful in differentiating ameloblastoma from ossifying fibroma, osteomyelitis, giant cell tumor, cystic fibrous dysplasia, myeloma, and sarcoma. The best treatment of ameloblastoma is aggressive en bloc resection with simultaneous reconstruction. The high recurrence rate and large tissue defects have been long-standing issues in the treatment of ameloblastoma. Recent molecular developments strongly suggest the possibility of targeted therapy with better outcomes in ameloblastomas. We present a detailed updated narrative review of our current understanding and management of this enigmatic tumor.

Keywords: adamantinoma; ameloblastoma; brafv600e mutation; jaw neoplasms; mandible; mandibular neoplasms; maxilla; maxillary neoplasms; multicystic ameloblastoma; odontogenic tumors.

Figure 1. Figure depicting four histologic subtypes of ameloblastoma. A: unicystic, B: follicular, C: plexiform, and D: desmoplastic

Reproduced with permission from McClary AC, West RB, McClary AC, et al. Ameloblastoma: a clinical review and trends in management. Eur Arch Oto-Rhino-Laryngol. 2016;273(7):1649-1661