On the Horizon for Supratentorial Primitive Neuroectodermal Tumors in Children

  • Ongoing assessment of efficacy of multimodal therapy: The roles of radiotherapy and chemotherapy are being reassessed. A simple search of the clinical trials registry reveals that studies continue to recruit supratentorial PNET patients in order to further refine understanding of the optimal sequence of treatment (radiation, then chemotherapy; radiation and chemotherapy, then consolidation chemotherapy and stem cell rescue) as well as to study investigational agents.
  • Radiotherapy: Improved local control may be effected with higher doses of radiation to the tumor bed. It is still poorly understood whether higher doses of local radiation might improve tumor control without the unacceptable long-term side effects. The use of hyperfractionated radiotherapy may allow for higher doses of radiation without the untoward damage to normal brain. Newer and more aggressive chemotherapeutic regimens, with higher drug doses with or without bone marrow rescue, may lead to better outcomes.
  • Molecular biology: Ultimately, a better understanding of the molecular biology of supratentorial PNET may hold future promise in providing more effective means of therapy for children with these tumors. There has been an incremental improvement in the understanding of the optimal treatments for this challenging disease, and it is hoped that continued study will continue to pay dividends for both the likelihood of survival and the quality of that survival.