title: Primitive neuroectodermal tumors in the central nervous system following cranial irradiation: a report of four cases.
abstract: BACKGROUND
  Radiation induced intracranial neoplasms are uncommon but well described and include gliomas, meningiomas, and sarcomas. The development of primitive neuroectodermal tumors (PNETs) following prophylactic craniospinal irradiation has been infrequently reported previously. The authors present four additional cases of PNETs that developed after previous cranial irradiation.
  
  
  METHODS
  Four patients who had previously been irradiated were determined to have PNETs of the central nervous system characterized by histopathologic and immunohistochemical features. The average patient age at diagnosis of the initial tumors and cranial irradiation was 17 years. The PNETs developed 5, 11, 11, and 18 years after completion of radiation.
  
  
  RESULTS
  Three patients had posterior fossa tumors, one pilocytic astrocytoma, one low grade astrocytoma, and one malignant ependymoma, which had been diagnosed and treated in childhood. Two of those patients developed supratentorial PNETs and the third a cerebellar hemispheric PNET. The fourth patient developed a posterior fossa PNET following irradiation for a temporal astrocytoma, which was initially diagnosed and resected at 37 years of age. Mean survival was 12 months after diagnosis.
  
  
  CONCLUSIONS
  The development of PNETs after cranial irradiation may be more common than thought previously and should be considered in the differential diagnosis of irradiation induced neoplasms. Survival after diagnosis of these radiation induced PNETs was short, and this may reflect an inability to provide standard therapy used for primary PNETs.
authors.affiliation: Division of Neurosurgery, Department of Clinical Neurosciences, University of Calgary, Calgary, Canada.
authors.forename: Walter J
authors.initials: WJ
authors.lastname: Hader
authors.forename: Katerina
authors.initials: K
authors.lastname: Drovini-Zis
authors.forename: John A
authors.initials: JA
authors.lastname: Maguire
mesh_terms: D000293:Adolescent; D000328:Adult; D001932:Brain Neoplasms / Q000209:etiology* / Q000532:radiotherapy; D002648:Child; D016371:Cranial Irradiation / Q000009:adverse effects*; D005260:Female; D006801:Humans; D008297:Male; D009381:Neoplasms, Radiation-Induced; D018242:Neuroectodermal Tumors, Primitive / Q000209:etiology*; D011878:Radiotherapy / Q000009:adverse effects