Primitive neuroectodermal tumor: Difference between revisions

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{{hatnote|Not to be confused with [[pancreatic neuroendocrine tumor]], which is also abbreviated as PNET.}}
{{Infobox medical condition |
{{Infobox_Disease |
Name = Primitive neuroectodermal tumor |
Image = PNET_Histopathology_HE_200x.jpg |
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'''Primitive neuroectodermal tumor''' (PNET) 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 = }}</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]].
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PNET of the CNS generally refer to supratentorial PNETs.
 
* In the past [[medulloblastoma]]s were considered PNETs; however, they are genetically, transcriptionally and clinically distinct. As such, "infratentorial" PNETs are now referred to as medulloblastoma {{Citation needed|date=March 2013}}.
 
* [[Pineoblastoma]]s are embryonal tumours originating in the [[pineal gland]] and are likely distinct from supratentorial PNETs.
 
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{{Osseous and chondromatous tumors}}
{{Small blue round cell tumors}}
 
[[Category:Pediatrics]]
[[Category:Rare cancers]]