Supratentorial Primitive Neuroectodermal Tumor

  • Mary Frances McAleerEmail author


Supratentorial primitive neuroectodermal tumors (SPNET) are rare small round blue cell malignancies with distinct genetic alterations and worse outcomes than the more common infratentorial PNET, medulloblastoma, that most often affects young children less than 5 years of age. Clinical presentation varies with tumor location, and these tumors are often identified by distinct radiographic features including abnormal diffusion restriction on diffusion-weighted magnetic resonance imaging and absence of peritumoral edema, despite the large size of many of these tumors at presentation. Standard therapy for patients with SPNET has been extrapolated from medulloblastoma regimens and combines maximal safe resection and adjuvant radiotherapy and chemotherapy. Outcomes of patients with SPNET have been shown to vary with tumor location (pineal vs. non-pineal), stage, extent of resection, and patient age. This chapter summarizes the data supporting current management strategies, with particular focus on role and delivery of radiation treatment, for pediatric patients with this rare, aggressive brain tumor.


Supratentorial primitive neuroectodermal tumor (SPNET) Pineoblastoma Pediatric Radiotherapy Combined modality therapy 


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Copyright information

© Springer International Publishing AG 2018

Authors and Affiliations

  1. 1.Department of Radiation OncologyThe University of Texas MD Anderson Cancer CenterHoustonUSA

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