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Primitive neuroectodermal tumour (view source)
Revision as of 13:44, 4 October 2017
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''' CNS Primitive neuroectodermal tumour''', abbreviated '''CNS-PNET''', is an | ''' 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 Since 2016 this category has been replaced by the designation '''other CNS embryonal tumors'''. | |||
==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]]''. | ||
* | *The former category contained a heterogenous group of poorly differentiated WHO grade IV tumours associated with following ICD-O codes: | ||
**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=== | |||
* Since WHO 2016 CNS classification this is now a subgroup of [[Other CNS embryonal tumours]]. | |||
* Many CNS neuroblastoma / CNS ganglioneuroblastoma cluster molecularly into a group designated as CNS NB-FOXR2.<ref>{{Cite journal | last1 = Sturm | first1 = D. | last2 = Orr | first2 = BA. | last3 = Toprak | first3 = UH. | last4 = Hovestadt | first4 = V. | last5 = Jones | first5 = DTW. | last6 = Capper | first6 = D. | last7 = Sill | first7 = M. | last8 = Buchhalter | first8 = I. | last9 = Northcott | first9 = PA. | title = New Brain Tumor Entities Emerge from Molecular Classification of CNS-PNETs. | journal = Cell | volume = 164 | issue = 5 | pages = 1060-1072 | month = Feb | year = 2016 | doi = 10.1016/j.cell.2016.01.015 | PMID = 26919435 }}</ref> | |||
*Usu Olig-2 +ve. | |||
*Synaptophysin +ve. | |||
===CNS ganglioneuroblastoma=== | |||
* This is now a subgroup of [[Other CNS embryonal tumours]]. | |||
===Lipomatous medulloblastoma=== | |||
* These tumors are now designated as [[Cerebellar liponeurocytoma]]. | |||
===Melanotic medulloblastoma=== | |||
* In WHO CNS 1997 still a distinct tumor. | |||
* These tumors are now considered a variant of [[medulloblastoma]]. | |||
===Medullomyoblastoma=== | |||
* Embryonal tumor with primitive neuronal cells and striated muscle component. | |||
* First description in 1933.<ref>{{Cite journal | last1 = Brody | first1 = BS. | last2 = German | first2 = WJ. | title = Medulloblastoma of the Cerebellum: A Report of 15 Cases. | journal = Yale J Biol Med | volume = 6 | issue = 1 | pages = 19-29 | month = Oct | year = 1933 | doi = | PMID = 21433586 }}</ref> | |||
* Since WHO 2007 CNS tumor classification tumors were classified as ''medulloblastoma with myogenic differentiation''. | |||
===Medulloepithelioma=== | ===Medulloepithelioma=== | ||