The rate of PNETs in not correlated with sex, but it shows a correlation with age. Most cases occur in children around 5 years of age, having a very low frequency in adults. Regarding genetic mutations, a specific type of gene alteration that directly leads to this tumor hasn't been defined yet. However, a positive correlation between individuals with Li-Fraumeni syndrome with a mutation in the gene p53 and PNET has been reported. A significant number of individuals with mutations on the rb tumor suppressor gene have also developed the tumor. Such gene encodes for the protein Rb responsible for stopping the cell cycle at the G1 phase. Another possible contributing factor are mutations in the CREB-binding protein, whose function includes activating transcription, but this interaction still need to be studied further. It has also been presumed that the tumor can arise from cranial irradiation.
Central nervous system primitive neuroectodermal tumor
Central nervous system cyst
Extracranial germ cell tumor
Pediatric ependymoma
Primary central nervous system lymphoma
Primitive neuroectodermal tumor
Glial tumor
Central neurocytoma