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{{Short description|Cancer of the developing nervous system}}
{{hatnote|Not to be confused with [[pancreatic neuroendocrine tumor]], which is also abbreviated as PNET.}}
{{Infobox medical condition (new)
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| caption = Micrograph of an H&E stained section of a peripheral PNET.
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| pronounce =
| field = Oncology
| synonyms = PNET
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'''Primitive neuroectodermal tumor''' is a malignant (cancerous) [[neural crest]] tumor.<ref>{{DorlandsDict|eight/000112249|primitive neuroectodermal tumor}}</ref> It is a rare [[tumor]], usually occurring in children and young adults under 25 years of age. The overall 5 year survival rate is about 53%.<ref name=Smoll20111>{{Cite journal | last1 = Smoll | first1 = N. R. | title = Relative survival of childhood and adult medulloblastomas and primitive neuroectodermal tumors (PNETs) | doi = 10.1002/cncr.26387 | journal = Cancer | volume = 118 | issue = 5 | pages = 1313–1322 | year = 2012 | pmid = 21837678| s2cid = 8490276 | doi-access = free }}</ref>
It gets its name because the majority of the cells in the tumor are derived from [[neuroectoderm]], but have not developed and differentiated in the way a normal [[neuron]] would, and so the cells appear "primitive". PNET belongs to the [[Ewing family of tumors]].
==Genetics==
Using gene transfer of SV40 large T-antigen in neuronal precursor cells of rats, a brain tumor model was established. The PNETs were histologically indistinguishable from the human counterparts and have been used to identify new genes involved in human brain tumor carcinogenesis.<ref name="pmid8129041">{{cite journal |vauthors=Eibl RH, Kleihues P, Jat PS, Wiestler OD |title=A model for primitive neuroectodermal tumors in transgenic neural transplants harboring the SV40 large T antigen |journal=Am. J. Pathol. |volume=144 |issue=3 |pages=556–64 |date=March 1994 |pmid=8129041 |pmc=1887088 }}</ref> The model was used to confirm p53 as one of the genes involved in human medulloblastomas, but since only about 10% of the human tumors showed mutations in that gene, the model can be used to identify the other binding partners of SV40 Large T- antigen, other than p53.<ref name="pmid1933879">{{cite journal |vauthors=Ohgaki H, Eibl RH, Wiestler OD, Yasargil MG, Newcomb EW, Kleihues P |title=p53 mutations in nonastrocytic human brain tumors |journal=Cancer Res. |volume=51 |issue=22 |pages=6202–5 |date=November 1991 |pmid=1933879 |url=http://cancerres.aacrjournals.org/cgi/pmidlookup?view=long&pmid=1933879}}</ref>{{additional citation needed|date=July 2021}}
==Diagnosis==
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