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Biological behavior of the primitive neuroectodermal tumors: significant supratentorial childhood gliomas.

Supratentorial gliomas in the pediatric age group at the University of Kentucky Medical Center accounted for 20% of all childhood brain tumors. Seventeen of the 20 children with these tumors, regardless of histologic type, mode of therapy, or other parameters died within five years after tissue diagnosis, and most were dead within two years. Unlike the astrocytic gliomas, the poorly differentiated primitive neuroectodermal tumors tended to spread diffusely throughout the central nervous system and accounted for 35% of the supratentorial gliomas in children at our institution. Results of this study suggest that therapy for the primitive neuroectodermal tumor, unlike other childhood supratentorial gliomas, should be considered for the entire neuraxis.