Difference between revisions of "Primitive neuroectodermal tumour"

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Primitive neuroectodermal tumour (view source)

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''' CNS Primitive neuroectodermal tumour''', abbreviated '''CNS-PNET''', is an uncommon [[neuropathology tumour]] in the group of embryonal tumours.
''' CNS Primitive neuroectodermal tumour''', abbreviated '''CNS-PNET''', is an abandoned [[neuropathology tumour]] description within in the group of embryonal tumours.
 
The terminology was introduced in 1973 <ref>{{Cite journal  | last1 = Hart | first1 = MN. | last2 = Earle | first2 = KM. | title = Primitive neuroectodermal tumors of the brain in children. | journal = Cancer | volume = 32 | issue = 4 | pages = 890-7 | month = Oct | year = 1973 | doi =  | PMID = 4751919 }}</ref> and used in the WHO 2007 classification of CNS tumors. Since 2016 this category has been replaced by the designation '''other CNS embryonal tumors'''.
It is also known as '' supratentorial primitive neuroepithelial tumour'' (supratentorial PNET).


==General==
==General==
*Should '''not''' be confused with ''peripheral primitive neuroectodermal tumour'' (abbreviated ''[[pPNET]]''<ref name=PST14feb11>PST. 14 February 2011.</ref>), [[AKA]] ''[[Ewing sarcoma]]''.
*Should '''not''' be confused with ''peripheral primitive neuroectodermal tumour'' (abbreviated ''[[pPNET]]''<ref name=PST14feb11>PST. 14 February 2011.</ref>), [[AKA]] ''[[Ewing sarcoma]]''.
*Currently contains a heterogenous group of poorly differentiated WHO grade IV tumours. '''Major reoganisation of this group will occur in the upcoming WHO classification.'''
*The former category contained a heterogenous group of poorly differentiated WHO grade IV tumours associated with following ICD-O codes:
*Mainly children and adolescents.
*Cerebral hemisphere, brain stem or spinal cord.
*Cerebrospinal dissemination found in up to 1/3 patients.<ref name="pmid1030655">{{Cite journal  | last1 = Horten | first1 = BC. | last2 = Rubinstein | first2 = LJ. | title = Primary cerebral neuroblastoma. A clinicopathological study of 35 cases. | journal = Brain | volume = 99 | issue = 4 | pages = 735-56 | month = Dec | year = 1976 | doi =  | PMID = 1030655 }}</ref>
*There are currently five ICD-O codes assigned within this group:
**9473/3 CNS-PNET, NOS.
**9473/3 CNS-PNET, NOS.
**9500/3 CNS neuroblastoma.
**9500/3 CNS neuroblastoma.
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**9501/3 Medulloepithelioma.
**9501/3 Medulloepithelioma.
**9392/3 Ependymoblastoma.
**9392/3 Ependymoblastoma.
*Mainly children and adolescents.
*Cerebral hemisphere, brain stem or spinal cord.
*Cerebrospinal dissemination found in up to 1/3 patients.<ref name="pmid1030655">{{Cite journal  | last1 = Horten | first1 = BC. | last2 = Rubinstein | first2 = LJ. | title = Primary cerebral neuroblastoma. A clinicopathological study of 35 cases. | journal = Brain | volume = 99 | issue = 4 | pages = 735-56 | month = Dec | year = 1976 | doi =  | PMID = 1030655 }}</ref>
* Very poor prognosis<ref name="pmid26304823">{{Cite journal  | last1 = Tulla | first1 = M. | last2 = Berthold | first2 = F. | last3 = Graf | first3 = N. | last4 = Rutkowski | first4 = S. | last5 = von Schweinitz | first5 = D. | last6 = Spix | first6 = C. | last7 = Kaatsch | first7 = P. | title = Incidence, Trends, and Survival of Children With Embryonal Tumors. | journal = Pediatrics | volume = 136 | issue = 3 | pages = e623-32 | month = Sep | year = 2015 | doi = 10.1542/peds.2015-0224 | PMID = 26304823 }}</ref>
* Very poor prognosis<ref name="pmid26304823">{{Cite journal  | last1 = Tulla | first1 = M. | last2 = Berthold | first2 = F. | last3 = Graf | first3 = N. | last4 = Rutkowski | first4 = S. | last5 = von Schweinitz | first5 = D. | last6 = Spix | first6 = C. | last7 = Kaatsch | first7 = P. | title = Incidence, Trends, and Survival of Children With Embryonal Tumors. | journal = Pediatrics | volume = 136 | issue = 3 | pages = e623-32 | month = Sep | year = 2015 | doi = 10.1542/peds.2015-0224 | PMID = 26304823 }}</ref>


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*Fibrillary background in tumours with advanced neuronal maturation (ganglioneuroblastomas).
*Fibrillary background in tumours with advanced neuronal maturation (ganglioneuroblastomas).
*Variable mitotic activity.
*Variable mitotic activity.
===Supratentorial PNET===
* This category of small round- and blue cell tumor was used in the WHO 2007 CNS tumor classification to separate them from medulloblastomas.
* Tumors are today classified as [[AT/RT]], [[Pineoblastoma]], [[ETMR]], H3F3A-mutated [[glioblastoma]] or CNS embryonal tumor, NOS.
===CNS neuroblastoma===
===CNS ganglioneuroblastoma===
===Lipomatous medulloblastoma===
===Medullomyoblastoma===


===Medulloepithelioma===
===Medulloepithelioma===
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