title: Dabrafenib in BRAFV600-mutated anaplastic pleomorphic xanthoastrocytoma.
abstract: Pleomorphic xanthoastrocytoma (PXA) is a rare brain tumor. Anaplastic features are found in 20-30% of cases of PXA and are associated with poor outcomes. Typical treatment is with gross total resection, followed by radiation therapy and cytotoxic chemotherapy at relapse. BRAFV600 mutations have been identified in 38-60% of patients with PXA. Several case reports and small case series have identified clinical benefit with BRAF inhibition in patients with BRAFV600-mutated PXA. We report the second published case of successful treatment with the BRAF inhibitor dabrafenib in a female patient with relapsed anaplastic PXA with a BRAFV600 mutation, and the first published case of dabrafinib treatment following intolerance to vemurafenib.
authors: Department of Oncology, University College London Hospitals, 235 Euston Road, London NW1 2BU, UK.|UCL Cancer Institute, University College London, 72 Huntley Street, London WC1E 6DD, UK.
authors: Nicholas F
authors: NF
authors: Brown
authors: UCL Cancer Institute, University College London, 72 Huntley Street, London WC1E 6DD, UK.
authors: Thomas
authors: T
authors: Carter
authors: Department of Oncology, University College London Hospitals, 235 Euston Road, London NW1 2BU, UK.|UCL Cancer Institute, University College London, 72 Huntley Street, London WC1E 6DD, UK.
authors: Paul
authors: P
authors: Mulholland
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use*; D001254:Astrocytoma / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000235:genetics*; D001932:Brain Neoplasms / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000235:genetics*; D005260:Female; D006801:Humans; D007093:Imidazoles / Q000627:therapeutic use*; D008279:Magnetic Resonance Imaging; D009154:Mutation / Q000235:genetics*; D010091:Oximes / Q000627:therapeutic use*; D048493:Proto-Oncogene Proteins B-raf / Q000235:genetics*; D055815:Young Adult
chemical_list: D000970:Antineoplastic Agents; D007093:Imidazoles; D010091:Oximes; C482119:BRAF protein, human; D048493:Proto-Oncogene Proteins B-raf; C561627:dabrafenib
keywords: BRAF inhibitor; BRAFV600 mutation; PXA; anaplastic; dabrafenib; pleomorphic xanthoastrocytoma; vemurafenib