Difference between revisions of "Primitive neuroectodermal tumour"

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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 Since 2016 this category has been replaced by the designation '''other CNS embryonal tumors'''.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==

*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.

**9500/3 CNS neuroblastoma.

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**9501/3 Medulloepithelioma.

**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>

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*Fibrillary background in tumours with advanced neuronal maturation (ganglioneuroblastomas).

*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===

Difference between revisions of "Primitive neuroectodermal tumour"

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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==
 *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.
 **9500/3 CNS neuroblastoma.
@@ Line 47: / Line 42: @@
 **9501/3 Medulloepithelioma.
 **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>
@@ Line 58: / Line 57: @@
 *Fibrillary background in tumours with advanced neuronal maturation (ganglioneuroblastomas).
 *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===