Refractory Ameloblastoma in the Maxilla: Clinical, Imaging, Histological, Surgical and Mutational Characterization: A Case Series

Author:

Bernaola-Paredes Wilber E.1ORCID,Albuja-Rivadeneira Estefani2,Novelli Franco2,Dalcin José Francisco3,Vartanian José Guilherme2,Kohler Hugo Fontan2,Pellizzon Antonio Cássio Assis1

Affiliation:

1. Department of Radiation Oncology, A.C. Camargo Cancer Center, Sao Paulo, Brazil

2. Department of Head and Neck and Otorhinolaryngology, A.C. Camargo Cancer Center, Sao Paulo, Brazil

3. Department of Anatomic Pathology, A.C. Camargo Cancer center, Sao Paulo, Brazil

Abstract

Study Design: Ameloblastoma is a benign odontogenic tumor of epithelial origin, with locally aggressive behavior; and due to its invasiveness has increased recurrence rates. It is more frequent in the mandible than the maxilla. Surgical treatment is performed with approaches ranging from conservative to radical surgery, either followed by microvascular reconstruction, or not. Adjuvant treatment has shown better local control in refractory cases. Multiple relapses are associated with BRAF gene mutation at codon 600. Objective: This case series aimed to describe the clinical, imaging, histopathological, therapeutic and mutational characterization of 3 patients with refractory ameloblastoma in the maxilla. Methods: Data of 3 patients were collected, and descriptions were provided of procedures such as clinical, imaging, surgical technique, histopathological subtype and molecular analysis for detection of BRAFV600E mutation. Results It was obtained a locoregional control after RT of two cases described. After BRAF mutation molecular analysis, no patient presented it. Conclusion: Surgery remains the gold standard for the treatment of ameloblastomas, even in refractory cases; however, an expanded approach to obtain free surgical margins, and reconstruction of the maxilla itself may be challenging. Adjuvant radiotherapy is still a controversial topic, but could favor reduction of the local recurrence rate in cases where the surgical margins are compromised after surgical resection. Further studies will be necessaries for analysis of the BRAFV600E mutation, for therapeutic purposes.

Funder

Conselho Nacional de Desenvolvimento Cientfico e Tecnolgico

Publisher

SAGE Publications

Subject

General Medicine

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