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==General== | ==General== | ||
Other CNS embryonal tumours are a group of primitive neuroeptithelial tumors that are to be classified in distinct molecular groups in the future. | Other CNS embryonal tumours are a group of primitive neuroeptithelial tumors that are to be classified in distinct molecular groups in the future. | ||
Currently it is a exlcusion criteria encompassing four morphological subgroups | |||
*CNS neuroblastomas | |||
**often infants. | |||
**Tumors can be huge. | |||
*CNS ganglioneuroblastomas. | |||
** often characterized by FOXR2 fusions: ''CNS neuroblastoma with FOXR2 activation (NB‐FOXR2)'' | |||
*CNS medulloepithelioma. | |||
** but absence of C19MC alteration, otherwise classified as [[ETMR]] | |||
* CNS embryonal tumour, NOS | |||
** Similarities to astroblastoma: ''High‐grade neuroepithelial tumours with MN1 alteration (HGNET‐MN1)'' | |||
** NUTM1-positive IHC: ''Ewing's sarcoma family tumour with CIC alteration (EFT‐CIC)'' | |||
** BCOR-positive IHC: ''HGNET BCOR‐altered neuroepithelial tumours'' | |||
Currently four distinct subtypes can be discriminated by molecular analysis: NB-FOXR2, HGNET-MN1, EFT-CIC and HGNET-BCOR.<ref>{{cite journal |authors=Sturm D, Orr BA, Toprak UH, Hovestadt V, Jones DTW, Capper D, Sill M, Buchhalter I, Northcott PA, Leis I, Ryzhova M, Koelsche C, Pfaff E, Allen SJ, Balasubramanian G, Worst BC, Pajtler KW, Brabetz S, Johann PD, Sahm F, Reimand J, Mackay A, Carvalho DM, Remke M, Phillips JJ, Perry A, Cowdrey C, Drissi R, Fouladi M, Giangaspero F, Łastowska M, Grajkowska W, Scheurlen W, Pietsch T, Hagel C, Gojo J, Lötsch D, Berger W, Slavc I, Haberler C, Jouvet A, Holm S, Hofer S, Prinz M, Keohane C, Fried I, Mawrin C, Scheie D, Mobley BC, Schniederjan MJ, Santi M, Buccoliero AM, Dahiya S, Kramm CM, von Bueren AO, von Hoff K, Rutkowski S, Herold-Mende C, Frühwald MC, Milde T, Hasselblatt M, Wesseling P, Rößler J, Schüller U, Ebinger M, Schittenhelm J, Frank S, Grobholz R, Vajtai I, Hans V, Schneppenheim R, Zitterbart K, Collins VP, Aronica E, Varlet P, Puget S, Dufour C, Grill J, Figarella-Branger D, Wolter M, Schuhmann MU, Shalaby T, Grotzer M, van Meter T, Monoranu CM, Felsberg J, Reifenberger G, Snuderl M, Forrester LA, Koster J, Versteeg R, Volckmann R, van Sluis P, Wolf S, Mikkelsen T, Gajjar A, Aldape K, Moore AS, Taylor MD, Jones C, Jabado N, Karajannis MA, Eils R, Schlesner M, Lichter P, von Deimling A, Pfister SM, Ellison DW, Korshunov A, Kool M |title=New Brain Tumor Entities Emerge from Molecular Classification of CNS-PNETs |journal=Cell |volume=164 |issue=5 |pages=1060–1072 |date=February 2016 |pmid=26919435 |pmc=5139621 |doi=10.1016/j.cell.2016.01.015 |url=}}</ref> | |||
==Histology== | |||
CNS neuroblastoma: | |||
*Groups of neurocytic cells. | |||
*Variable neuropil-rich stroma. | |||
*Small round blue cells. | |||
DDx: | HGNET-BCOR: | ||
*Circumscribed growth. | |||
*Perivascular pesudorosettes. | |||
*Fibrillary areas resembling glioma. | |||
*Palisading necrosis. | |||
*Absence of microvascular proliferation. | |||
==IHC== | |||
CNS Ganglioneuroblastoma + Neuroblastoma | |||
* Syn -/+ve. | |||
* Vim -ve. | |||
* GFAP usu -ve. | |||
* MIB-1 usu high. | |||
Medulloepthelioma | |||
* Syn +/-ve. | |||
* NF +/-ve. | |||
* GFAP usu -ve. | |||
* CK may be focally +ve. | |||
HGNET-BCOR <ref>{{cite journal |authors=Ferris SP, Velazquez Vega J, Aboian M, Lee JC, Van Ziffle J, Onodera C, Grenert JP, Saunders T, Chen YY, Banerjee A, Kline CN, Gupta N, Raffel C, Samuel D, Ruiz-Diaz I, Magaki S, Wilson D, Neltner J, Al-Hajri Z, Phillips JJ, Pekmezci M, Bollen AW, Tihan T, Schniederjan M, Cha S, Perry A, Solomon DA |title=High-grade neuroepithelial tumor with BCOR exon 15 internal tandem duplication-a comprehensive clinical, radiographic, pathologic, and genomic analysis |journal=Brain Pathol. |volume=30 |issue=1 |pages=46–62 |date=January 2020 |pmid=31104347 |doi=10.1111/bpa.12747 |url=}}</ref> | |||
* OLIG variable +ve. | |||
* BCOR +ve. | |||
* Syn -ve. | |||
* GFAP -ve. | |||
* EMA: dots +ve. | |||
* NEUN +/-ve. | |||
==DDx:== | |||
* [[AT/RT]] | * [[AT/RT]] | ||
* [[ETMR]] | * [[ETMR]] | ||
* [[Anaplastic ependymoma]] | * [[Anaplastic ependymoma]] | ||
* H3F3A G34-mutated [[Glioblastoma]] | * H3F3A G34-mutated [[Glioblastoma]] | ||
* Metastasis of [[Ewing sarcoma]] | |||
[[Category:Neuropathology]] | |||
[[Category:Neuropathology tumours]] | |||