Adjuvant chemotherapy

The chemotherapeutic approaches during and after radiation therapy are similar to those used for children with high-risk medulloblastoma. The 3-year to 5-year OS rates range from 25% to 50%.[29-31]; [32,33][Level of evidence B4]; [34][Level of evidence C1]

In a published study of nonpineal tumors that were diagnosed as CNS primitive neuroectodermal tumors (PNETs) by traditional pathology, 71% of cases were revealed to be glioblastoma or another diagnosis by DNA methylation studies. Patients with nonmedulloblastoma embryonal tumors (n = 36) (including pineoblastomas, n = 26) had a 5-year OS rate of 78.5% (95% confidence interval [CI], 62.2%–94.8%). In contrast, the patients with glioblastoma had a 5-year OS rate of 12% (95% CI, 0%–24.7%). The study showed no benefit for children who received carboplatin or isotretinoin.[3][Level of evidence A1] This study highlights the importance of molecular classification of tumors traditionally termed CNS-PNET.[4]