Glioblastoma multiforme (GBM) is the most common and aggressive glial 
cell-derived primary tumor. Current standard of care for patients with GBM 
includes maximal tumor resection plus adjuvant radiotherapy and temozolomide 
chemotherapy, increasing median overall survival to a mere 15 months from 
diagnosis. Because these therapies are inherently nonspecific, there is an 
increased likelihood of off-target and incomplete effects; therefore, targeted 
modalities are required for enhanced safety and efficacy. Rindopepimut is 
emerging as a safe and potentially effective drug for the treatment of GBM. 
Rindopepimut consists of a 14-mer peptide that spans the length of EGF receptor 
variant III, a mutant variant of EGF receptor found on approximately 30% of 
primary GBM, conjugated to the carrier protein keyhole limpet hemocyanin. 
Vaccination with rindopepimut has been shown to specifically eliminate cells 
expressing EGF receptor variant III. Phase II clinical trials have suggested 
that vaccination of newly diagnosed GBM patients with rindopepimut plus adjuvant 
granulocyte-macrophage colony-stimulating factor results in prolonged 
progression-free and overall survival with minimal toxicity. This review will 
outline the development of rindopepimut, as well as the current status of this 
vaccine.