Cerebral primitive neuroectodermal tumors in childhood

A series of 17 infants and children with cerebral primitive neuroectodermal tumors (PNETs) detected by computed tomography is presented. The pertinent literature is reviewed. Because of ongoing nosological difficulty, we include in this series only those tumors which are located in the cerebral hemisphere, and are composed of predominantly undifferentiated neuroepithelial tumor with or without glial or neuronal differentiation. The prognosis of the patients with cerebral PNETs remains poor despite treatment including surgical resection, radiation therapy and/or chemotherapy. Only two patients had a long term survival more than 4 years in this series. The histological features and the extent of surgical resection did not influence the patients survival. Nevertheless, visible total resection afforded better control of local disease of the primary site, but remote metastases along the cerebrospinal fluid pathway were frequent at relapse. The patients with crebral PNETs appear to be best treated with radical gross total resection, postoperative radiation therapy including irradiation to the neuraxis and aggressive chemotherapy.