Objective:

To determine by systematic review the level of evidence available in terms of the diagnosis, prognosis, and treatment of BRAFV600E mutant ameloblastomas, delving into the possible benefit of molecular chemotherapy directed at the activating V600E genetic alteration in the BRAF protooncogene, without additional mutations, and in neoadjuvant scheme.

Materials and methods:

Based on the PRISMA guideline, a systematic bibliographic search was carried out in scientific databases of clinical studies published in the last 10 years on the prognostic, diagnosis and treatment of BRAFV600E mutant ameloblastomas.

Results:

The diagnosis of a BRAFV600E ameloblastoma is guided by clinical-radiographic criteria, reinforced with pathological criteria, and confirmed with molecular biology aimed at determining the V600E variant, which, according to strong evidence, is related to greater disease-free survival. Molecularly targeted chemotherapy, with an exclusive BRAF inhibitor, appears to offer a benefit over chemotherapy directed at two molecular targets of the MAPK pathway for recurrent or metastatic cases.

Conclusion:

The clinical and therapeutic management of BRAFV600E mutant ameloblastomas requires a multidisciplinary approach. Treatment with exclusive inhibitor in neoadjuvant therapy demonstrates survival advantages, and is reserved for recurrent or metastatic cases, although the evidence is deficient in quantity and quality.

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