Ameloblastoma is a benign, locally aggressive
neoplasm that needs extensive surgical resection. The goal of this article is to obtain an in-depth review of benign
ameloblastomas to determine the available level of evidence and the possible benefit of targeted
therapeutics for the treatment of
ameloblastoma and BRAF V600E mutation in
ameloblastoma. An electronic literature search was conducted according to
PRISMA guidelines in PubMed/MEDLINE, EBSCO, and Web of Science for eligible studies published between 1975 and 2021. The systematic review is registered with INPLASY (INPLASY202260018). The review included 2 case series and 17 case reports. The histopathological type, anatomic location, expression of BRAF mutation, additional mutations, and
molecular-targeted therapies of the 19 reviewed articles were summarized and tabulated. Interestingly, the majority of the primary site of
ameloblastoma was located in the mandible (80.9%) compared to the maxilla (17%). The tumour size was reported in nine of the included studies. Most of the included studies in the review exhibited
ameloblastoma with BRAF V600E mutations and responded to
molecular-targeted therapies. Molecular
therapies employing BRAF and/or
MEK inhibitors in
ameloblastoma with BRAF V600E mutations proved to be an appropriate treatment based on the limited available evidence. It is essential further to deepen our understanding at the clinical and molecular level to enhance the precision of management of
ameloblastoma.