Persistent response to vemurafenib in metastatic ameloblastoma with BRAF mutation: a case report

Ameloblastomas are uncommon locally aggressive tumors of odontogenic epithelium that rarely metastasize. Currently, there is no standard of care for the metastatic forms. Several studies have shown that ameloblastomas frequently have a BRAF mutation. We report a case of a 33-year-old Caucasian woman...

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Bibliographic Details
Published in:Journal of medical case reports 2019-07, Vol.13 (1), p.245-245, Article 245
Main Authors: Broudic-Guibert, Morgane, Blay, Jean-Yves, Vazquez, Léa, Evrard, Alexandre, Karanian, Marie, Taïeb, Sophie, Hoog-Labouret, Natalie, Oukhatar, Céline Mahier Ait, Boustany-Grenier, Rania, Arnaud, Antoine
Format: Article
Language:English
Subjects:
Adult
Ameloblastoma
Ameloblastoma - drug therapy
Ameloblastoma - pathology
Ameloblastoma - secondary
BRAF
Cancer
Cancer metastasis
Case Report
Case studies
Clinical trials
Drug therapy
Epithelium
Female
Gene mutation
Head and neck tumors
Health aspects
Humans
Jaw diseases
Jaw Neoplasms - surgery
Life Sciences
Lung Neoplasms - drug therapy
Lung Neoplasms - pathology
Lung Neoplasms - secondary
Lung Neoplasms / pahology
Metastatic
Mutation
Patient outcomes
Pharmaceutical sciences
Protein Kinase Inhibitors - administration & dosage
Proto-Oncogene Proteins B-raf
Tumors
Vemurafenib
Vemurafenib - administration & dosage
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Summary:Ameloblastomas are uncommon locally aggressive tumors of odontogenic epithelium that rarely metastasize. Currently, there is no standard of care for the metastatic forms. Several studies have shown that ameloblastomas frequently have a BRAF mutation. We report a case of a 33-year-old Caucasian woman with ameloblastoma diagnosed 30 years ago who developed lung metastasis 19 years ago. Systemic oral treatment with vemurafenib, a BRAF inhibitor, was initiated 28 months ago within the AcSé French basket clinical trial of vemurafenib. The patient has shown a durable clinical, functional, and radiographic partial response with vemurafenib. These observations suggest the possibility of introducing neoadjuvant and/or adjuvant targeted therapy in locally advanced ameloblastoma to improve outcome. BRAF inhibition has proved to be an efficient strategy in patients with a BRAF-mutated ameloblastoma.
ISSN:1752-1947
1752-1947
DOI:10.1186/s13256-019-2140-6