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

Journal of Medical Case Reports

Natalie Hoog-Labouret¹  Marie Karanian²  Jean-Yves Blay²  Sophie Taïeb³  Léa Vazquez⁴  Antoine Arnaud⁴  Morgane Broudic-Guibert⁴  Rania Boustany-Grenier⁴  Céline Mahier Ait Oukhatar⁵  Alexandre Evrard⁶ 

[1] INCA;Léon Bérard Center, University Claude Bernard Lyon 1;Oscar Lambret Center;Sainte-Catherine Institut;UNICANCER;University Hospital Center Carémeau;

关键词: Vemurafenib;    BRAF;    Ameloblastoma;    Metastatic;    Case report;   

DOI  :  10.1186/s13256-019-2140-6

来源: DOAJ

【 摘 要 】

Abstract Background 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. Case presentation 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. Conclusions 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.

【 授权许可】

Unknown