Primitive neuroectodermal tumor: Difference between revisions

Revision as of 19:05, 21 September 2020

Revision as of 08:57, 6 December 2020

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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| pmc = | s2cid = 8490276 }}</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".

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==Genetics==

{{More medical citations needed|section|date=March 2018}}

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 |doi= |url=}}</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 |doi= |url=http://cancerres.aacrjournals.org/cgi/pmidlookup?view=long&pmid=1933879}}</ref>

==Diagnosis==