Non-cerebellar primitive neuroectodermal tumors (PNET): Summary of the Milan consensus and state of the art workshop on marrow ablative chemotherapy with hematopoietic cell rescue for malignant brain tumors of childhood and adolescents

Abstract

CNS non-cerebellar primitive neuroectodermal tumors (PNET) represent <5% of all childhood brain tumors. Despite similar therapies, these patients have had significantly worse outcomes compared to patients with medulloblastoma. Although these tumors have historically been considered analogous to medulloblastoma, there is growing evidence that they are biologically distinct. Since these tumors are relatively rare, there are few large series in the literature. Here we present the international experience treating these patients with a variety of therapies, including marrow ablative chemotherapy followed by autologous hematopoietic cell rescue.