title: Systemic therapy for recurrent meningioma.
abstract: Meningioma comprise 20-30% of all primary brain tumors. Notwithstanding surgery and radiotherapy, a subset of patients will manifest recurrent meningioma. Systemic therapy is recommended only when further surgery and radiotherapy are not possible. No prospective study with a high level of evidence is available to inform as to recommendations regarding systemic therapy.
  
  
  
  We aim to summarize systemic therapies for recurrent meningioma. Expert commentary: Hydroxurea, temozolomide, irinotecan, the combination of cyclophosphamide/adriamycine/vincristine, interferon-alpha, somatostatin analogs, mifepristone, megestrol acetate, imatinib, erlotinib and gefitinib are considered as having limited efficacy. Potential activity of VEGF (vascular endothelial growth factor) inhibitors such as sunitinib, valatinib, and bevacizumab is suggested in small non-controlled studies and requires validation in randomized trials. The identification of new prognostic markers such as TERT promoter mutations and potential new therapeutic targets, such as KLF4, AKT1, TRAF7, and SMO mutations hopefully facilitate this endeavor.
authors.affiliation: a Lille University, PRISM Inserm U1191 , Villeneuve d'Ascq , France.|b Neuro-oncology, Department of Neurosurgery , Lille Universisty Hospital , Lille Cedex , France.|c Breast unit, Department of Medical Oncology , Oscar Lambret Center , Lille Cedex , France.
authors.forename: E
authors.initials: E
authors.lastname: Le Rhun
authors.affiliation: d Department of Neurology Mazarin , Pitié-Salpétrière Hospital, Assistance Publique des Hôpitaux de Paris , Paris , France.|e Department of Neurology , University Pierre et Marie Curie, Paris VI , Paris , France.
authors.forename: S
authors.initials: S
authors.lastname: Taillibert
authors.affiliation: f Department of Neurology and Neurological Surgery , University of Washington , Seattle , WA , USA.
authors.forename: M C
authors.initials: MC
authors.lastname: Chamberlain
mesh_terms: D000077156:Gefitinib; D006801:Humans; D000090062:Kruppel-Like Factor 4; D008577:Meningeal Neoplasms; D008579:Meningioma / Q000188:drug therapy*; D011446:Prospective Studies; D011799:Quinazolines; D042461:Vascular Endothelial Growth Factor A
chemical_list: C000715467:KLF4 protein, human; D000090062:Kruppel-Like Factor 4; D011799:Quinazolines; D042461:Vascular Endothelial Growth Factor A; D000077156:Gefitinib
keywords: Meningioma; bevacizumab; hydroyxurea; sunitinib; systemic therapy; trabectedin; valitinib