2016 Volume 53 Issue 5 Pages 481-486
An 11-year-old boy was transferred to our hospital because of headache and nausea. Cephalic MRI revealed a tumor in the left frontal lobe. Tumorectomy with subtotal removal was performed. A histopathological diagnosis of central nervous system primitive neuroectodermal tumor (CNS-PNET) was carried out. Chemo-radiotherapy followed by high-dose chemotherapy with stem cell rescue was conducted. However, the tumor increased in size. As severe paralysis was a concern, surgery was not indicated. Therefore, outpatient chemotherapy with vinorelbine and cyclophosphamide was started. There were no serious adverse events except for grade 3 peripheral neuropathy and grade 4 neutropenia, which required dose reduction of VNR. Subsequently, he has been well without CNS-PNET progression. The efficacy of VNR for solid tumors in children has been internationally reported. No standard treatment for refractory or relapsed CNS-PNET has been established. Further studies are needed to improve the outcome of refractory or relapsed CNS-PNET.