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{{Infobox medical condition
The Central Nervous System Primitive Neuroectodermal Tumor, often abbreviated as PNET, supratentorial PNET, or CNS-PNET,<ref name=":0">{{Cite journal|date=2015|editor-last=Karajannis|editor-first=Matthias A.|editor2-last=Zagzag|editor2-first=David|title=Molecular Pathology of Nervous System Tumors|url=http://dx.doi.org/10.1007/978-1-4939-1830-0|journal=Molecular Pathology Library|doi=10.1007/978-1-4939-1830-0|issn=1935-987X}}</ref> is one of the 3 types of embryonal central nervous system tumors defined by the [[World Health Organization]] ([[medulloblastoma]], [[Atypical teratoid rhabdoid tumor|atypical teratoid rhabdoid tumor]], and PNET).<ref name=":1">{{Cite journal|date=2014|editor-last=Hayat|editor-first=M.A.|title=Tumors of the Central Nervous System, Volume 13|url=http://dx.doi.org/10.1007/978-94-007-7602-9|journal=Tumors of the Central Nervous System|doi=10.1007/978-94-007-7602-9|issn=2215-096X}}</ref> It is considered an embryonal tumor because it arises from cells still undifferentiated from birth or partially differentiated .<ref name=":0" /> Those cells are usually [[Neuroepithelial cell|neuroepithelial cells]],<ref name=":0" /><ref name=":1" /><ref name=":2">{{Citation|last=Fuller|first=Christine E.|title=Oligodendroglial Tumors|date=2009-10-23|url=http://dx.doi.org/10.1007/978-1-4419-1062-2_4|work=Atlas of Pediatric Brain Tumors|pages=39–46|publisher=Springer New York|isbn=9781441910615|access-date=2019-02-24}}</ref> stem cells destined to turn into [[glia]] or [[Neuron|neurons]].<ref name=":3">{{Cite journal|last=Nelesen|first=Richard A|date=2000-03|title=Biological Psychology: An Introduction to Behavioral, Cognitive, and Clinical Neuroscience, 2nd edition. Mark R. Rosenweig, Arnold L. Leiman, and S. Marc Breedlove, Sinauer Associates, Inc., Sunderland MA, 1999. 561+92 pp. ISBN 0-87893-791-9|url=http://dx.doi.org/10.1016/s0301-0511(99)00025-3|journal=Biological Psychology|volume=52|issue=2|pages=185–186|doi=10.1016/s0301-0511(99)00025-3|issn=0301-0511}}</ref> It can occur anywhere within the [[Spinal cord|spinal cord]] and [[cerebrum]] and can have multiple sites of origins, with a high probability of [[metastasis]] through [[Cerebrospinal fluid|cerebrospinal fluid]] (CSF).<ref name=":0" /><ref name=":1" /> ▼
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|caption = Primitive neuroectodermal tumor of the [[central nervous system]] in a 5-year-old
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PNETs have five subtypes of tumors: neuroblastoma, ganglioneuroblastoma, medulloepithelioma, ependymoblastoma, and not otherwise specified PNET.<ref name=":0" /> They are similar to medulloblastomas in regards to histology but different in regards to site of tumor and genetic factors. It is a rare tumor occurring mostly among children, <ref name=":0" /><ref name=":1" /> accounting for 1.9 to 7% of childhood brain tumors.<ref name=":1" /> Its diagnosis can be acquired through magnetic resonance imaging (MRI) or computed tomography (CT).<ref name=":1" /> There isn't a standardized procedure, but common strategies involve chemotherapy and radiotherapy for individuals older than 3 years of age.<ref name=":0" /><ref name=":1" /> Their effectiveness is still controversial.<ref name=":1" /> Surgery is used to remove mass affected by the tumorous cells.<ref name=":1" /> The prognosis of the disease is more positive for adults than for children, who have a higher probability of developing side effects.<ref name=":0" /><ref name=":1" />▼
▲
== Classification ==▼
[[File:Medulloepithelioma Histology.jpg|thumb|Histology of Medulloepithelioma]]▼
The [[World Health Organization]] has classified the Central Nervous System Primitive Neuroectodermal Tumor into 5 subtypes: [[neuroblastoma]], [[ganglioneuroblastoma]], [[medulloepithelioma]], [[Primitive neuroectodermal tumor|ependymoblastoma]], and not otherwise specified PNET.<ref name=":0" /> The last one encompasses the PNETs with varying characteristics that hasn't been well defined yet.<ref name=":0" /> [[Neuroblastoma|Neuroblastomas]] are PNETS that involve the process of cell differentiation into neurons,<ref name=":0" /><ref name=":1" /> while [[Ganglioneuroblastoma|ganglioneuroblastomas]] are PNETs that involve ganglion cells.<ref name=":0" /> [[Medulloepithelioma]], on the other hand, are tumors involving the constant cell division on the [[epithelium]] tissue where bundle of neuron endings are located.<ref name=":0" /> Such tissue will differentiate into a similar form as the embryonic neural tube, also known as the starting structure of the [[Central nervous system|central nervous system]]. <ref name=":0" /><ref name=":1" /><ref name=":2" /> [[Medulloepithelioma|Medulloepitheliomas]] also present a pattern known as rosettes, characterized by the arrangement of a bundle of cells into circular shapes and around a center or a neuropil.<ref name=":0" /> [[Primitive neuroectodermal tumor|Ependymoblastoma]] also present rosettes as well as a higher density of cells.<ref name=":0" /><ref name=":2" /> It involves the process of differentiation into ependymal cells.<ref name=":1" /><ref name=":2" />▼
[[File:Ependymoblastomatous Rosette.jpg|thumb|Rosettes in Ependymoblastoma histology]]▼
Further classification types have come up but not yet approved by the [[World Health Organization]].<ref name=":0" /> The term "embryonal tumor with abundant neuropil and true rosettes", or ETANTR, has been proposed as a sixth subtype of PNET.<ref name=":0" /> However, the still unofficial term "embryonal tumor with multilayered rosettes" (ETMR) has been more frequently used and encompasses ETANTRs, [[Medulloepithelioma|medulloepitheliomas]], [[Primitive neuroectodermal tumor|ependymoblastomas]], and variants of PNETs with presence of rosettes and with no well defined classification.<ref name=":2" />▼
It is important to note that this classification term has been removed from the latest WHO classification of CNS tumors as of 2016. Instead PNETs are now included into the category of "Embryonal Tumors with Multilayered Rosettes" along with ependymoblastoma and embryonal tumor with abundant neuropil and true rosettes (ETANTR).<ref>Louis DN, Ohgaki H, Wiestler OD, Cavenee WK "WHO Classification of Tumours of the Central Nervous System. 4th Edition Revised"</ref>
▲== Classification ==
▲[[File:Medulloepithelioma Histology.jpg|thumb|Histology of [[Medulloepithelioma]]]]
The [[World Health Organization]] has classified the central nervous system primitive neuroectodermal tumors into five subtypes: [[neuroblastoma]], [[ganglioneuroblastoma]], [[medulloepithelioma]], ependymoblastoma, and not otherwise specified PNET.<ref name=":0" /> The last one encompasses the PNETs with varying characteristics that hasn't been well defined yet.<ref name=":0" /> [[Neuroblastoma]]s are PNETS that involve the process of cell differentiation into neurons,<ref name=":0" /><ref name=":1" /> while [[ganglioneuroblastoma]]s are PNETs that involve [[ganglion cell]]s.<ref name=":0" />
▲
== Risk Factors ==▼
▲[[File:Ependymoblastomatous Rosette.jpg|thumb|Rosettes in Ependymoblastoma histology]]
The rate of PNETs in not correlated with sex, but it is correlated with age.<ref name=":0" /> Most cases occur on children around 5 years of age while the frequency of PNETs in adults is very low. <ref name=":0" /> Regarding genetic mutations, a specific type of gene alteration that directly leads to this tumor hasn't been defined. <ref name=":0" /> However, a positive correlation between individuals with [[Li–Fraumeni syndrome|Li-Fraumeni syndrome]] with a mutation in the [[P53|gene ''p53'']] and PNET has been reported.<ref name=":1" /> A significant number of individuals with mutations on the [[Retinoblastoma protein|''rb'' tumor supressor gene]], encoding for the protein Rb responsible for stopping the cell cycle at the [[G1 phase]]<ref name=":4">{{Cite journal|last=Baker|first=Henry V|date=2003-06|title=Essential Genetics: A Genomics Perspective. Third Edition. By Daniel L Hartl and , Elizabeth W Jones. Sudbury (Massachusetts): Jones and Bartlett Publishers. $78.95 (paper). xxvi + 613 p; ill.; index. ISBN: 0–7637–1852–1. 2002.|url=http://dx.doi.org/10.1086/377959|journal=The Quarterly Review of Biology|volume=78|issue=2|pages=225–226|doi=10.1086/377959|issn=0033-5770}}</ref>, have also developed the tumor.<ref name=":1" /> Another possible contributing factor are mutations in the [[CREB-binding protein]] CBP, whose function includes activating transcription,<ref name=":4" /> but this interaction still need to be studied further.<ref name=":1" /> It has also been presumed that the tumor can arise from cranial irradiation.<ref name=":1" />▼
▲Further classification types have come up but not yet approved by the [[World Health Organization]].<ref name=":0" /> The term "embryonal tumor with abundant neuropil and true rosettes", or ETANTR, has been proposed as a sixth subtype of PNET.<ref name=":0" /> However, the still unofficial term "embryonal tumor with multilayered rosettes" (ETMR) has been more frequently used and encompasses ETANTRs, [[
▲The rate of PNETs in not correlated with sex, but it
== Diagnosis ==
[[File:MRI of PNET.jpg|thumb|[[Magnetic resonance imaging|Magnetic resonance]] image of PNET]]
Most children that develop primitive neuroectodermal tumors are diagnosed early in life, usually at around
Several analysis can
== Treatment ==
There is not a standardized procedure to treat primitive neuroectodermal tumors.<ref name=":1" />
A method for eliminating tumorous mass is surgery, where the best outcome would be total resection, meaning the complete removal of the tumor.<ref name=":1" /> Along with the surgery, several measures that contribute to a safe procedure can be taken: urine exams, transfusion, and the constant supervision of arterial pressure.<ref name=":1" /> Possible problems that arise from the surgery include [[Bleeding|hemorrhage]], [[Cerebral edema|brain edema]], and [[hemiparesis]].<ref name=":1" /> [[Magnetic resonance imaging|MRIs]] are typically done after 1 or 2 days of postoperative in order to inspect the amount of tumor remaining.<ref name=":1" />
== Prognosis ==
The probability of primitive neuroectodermal tumors to have recurrence and [[Metastasis|metastasize]] through [[
The effect of treatment strategies such as [[chemotherapy]] and [[
▲The effect of treatment strategies such as [[chemotherapy]] and [[Radiation therapy|radiation therapy]] on the prognosis of the disease is still controversial, with studies claiming either their benefits or their ineffectiveness.<ref name=":1" /> The same holds true for the relationship between volume of tumor removed by surgery and survival.<ref name=":1" /> Furthermore, factors such as tumor size, ___location of origin, race, and sex of individual don't show any influence on the outcome of the disease.<ref name=":1" /> However, interactions of some factors such as tumor site, age, and treatment strategy can affect one's prognosis.<ref name=":1" /> For instance, when younger children below the age of 3 suffering from tumors originating in places other than the [[Pineal gland|pineal gland]] are treated with [[chemotherapy]], they present better outcomes than those suffering from [[Pineal gland|pineal]] tumors.<ref name=":1" />
== References ==
{{Reflist}}
[[Category:Nervous system neoplasia]]
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