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* Anaplastic ependymoma |
==Choroid plexus tumours== |
* [[Choroid plexus papilloma]] |
* Atypical choroid plexus papilloma |
* [[Choroid plexus carcinoma]] |
==Other neuroepithelial tumours== |
* [[Neuropathology_tumours#Cribiform_neuroepithelial_tumour|Cribifiorm neuroepithelial tumour]]. |
===Cribiform neuroepithelial tumour=== |
AKA: '''CRINET'''. |
*Not listed in the current WHO classification. |
*First description in 2009.<ref>{{Cite journal | last1 = Hasselblatt | first1 = M. | last2 = Oyen | first2 = F. | last3 = Gesk | first3 = S. | last4 = Kordes | first4 = U. | last5 = Wrede | first5 = B. | last6 = Bergmann | first6 = M. | last7 = Schmid | first7 = H. | last8 = Frühwald | first8 = MC. | last9 = Schneppen... |
*Around ventricles.<ref>{{Cite journal | last1 = Arnold | first1 = MA. | last2 = Stallings-Archer | first2 = K. | last3 = Marlin | first3 = E. | last4 = Grondin | first4 = R. | last5 = Olshefski | first5 = R. | last6 = Biegel | first6 = JA. | last7 = Pierson | first7 = CR. | title = Cribriform neuroepithelial tumor ar... |
*Young children.<ref>{{Cite journal | last1 = Park | first1 = JY. | last2 = Kim | first2 = E. | last3 = Kim | first3 = DW. | last4 = Chang | first4 = HW. | last5 = Kim | first5 = SP. | title = Cribriform neuroepithelial tumor in the third ventricle: a case report and literature review. | journal = Neuropathology | vol... |
*Small undifferentiated cells arranged in cribriform strands and trabeculae of varying thickness. |
*MAP2+ve, Synaptophysin+ve, CK+/-ve. MIB-1: 30%. |
*INI-1 loss, but no rhabdoid features and good prognosis. |
*Stable genomic profile.<ref>{{Cite journal | last1 = Gessi | first1 = M. | last2 = Japp | first2 = AS. | last3 = Dreschmann | first3 = V. | last4 = Zur Mühlen | first4 = A. | last5 = Goschzik | first5 = T. | last6 = Dörner | first6 = E. | last7 = Pietsch | first7 = T. | title = High-Resolution Genomic Analysis of Cri... |
==Neuronal and mixed neuronal/glial tumours== |
* [[Desmoplastic infantile astrocytoma]] / ganglioglioma (DIA/DIG) |
* [[Dysembryoplastic neuroepithelial tumour]] |
* [[Central Neurocytoma]] / Extraventricular [[neurocytoma]] |
* Cerebellar liponeurocytoma |
* [[Papillary glioneuronal tumour]] (PGNT) |
* [[Rosette-forming glioneuronal tumour of the fourth ventricle]] (RGNT) |
* Gangliocytoma / Ganglioglioma |
* Dysplastic ganglioglioma of the cerebellum ([[Lhermitte-Duclos disease]]) |
* [[Paraganglioma]] |
===Desmoplastic infantile astrocytoma / Desmoplastic infantile ganglioglioma=== |
* Abbreviated ''DIA'' or ''DIG''. |
* ICD-O code: 9412/1 |
* Large, superficial, cystic tumor of the infancy. |
* Biologic course corresponds to WHO grade I. |
* Very rare, included in the WHO since 1993. |
* Prominent desmoplastic stroma. |
* Astrocytic cells within stroma. |
**GFAP+. |
**MIB-1 usu. 1%. |
* Frequent BRAF V600E or V600D mutations.<ref>{{Cite journal | last1 = Wang | first1 = AC. | last2 = Jones | first2 = DTW. | last3 = Abecassis | first3 = IJ. | last4 = Cole | first4 = BL. | last5 = Leary | first5 = SES. | last6 = Lockwood | first6 = CM. | last7 = Chavez | first7 = L. | last8 = Capper | first8 = D. | l... |
*Single case with BRAF indel or BRAF fusion. |
<gallery> |
File:DIG-histology.jpg | Histopathology of DIG (HE stain) |
File:DIG-histology2.jpg | Prominent ganglioid cells in DIG (HE stain) |
</gallery> |
===Cerebellar liponeurocytoma=== |
* Previously called ''lipomatous medulloblastoma'' (name changed in WHO 2000). |
* Mean age: 50 years. |
* As the name states: A tumour of the cerebellum. |
** But cases outside cerebellum reported that would qualify.<ref>{{Cite journal | last1 = Gupta | first1 = K. | last2 = Salunke | first2 = P. | last3 = Kalra | first3 = I. | last4 = Vasishta | first4 = RK. | title = Central liponeurocytoma: case report and review of literature. | journal = Clin Neuropathol | volume = ... |
* WHO grade II <ref>{{Cite journal | last1 = Nishimoto | first1 = T. | last2 = Kaya | first2 = B. | title = Cerebellar liponeurocytoma. | journal = Arch Pathol Lab Med | volume = 136 | issue = 8 | pages = 965-9 | month = Aug | year = 2012 | doi = 10.5858/arpa.2011-0337-RS | PMID = 22849747 }}</ref> (upgraded from WHO ... |
*ICD-O code: 9506/1 |
====Histo==== |
* Advanced neuronal and lipomatous differentiation. |
* Neurocytes: round to oval nuclei with clear cytoplasm. |
* Quite cellular. |
* Mitoses almost absent. |
====IHC==== |
* [[GFAP]] +/-ve (focal). |
* [[MAP2]] +ve. |
* Synaptophysin +ve. |
* NeuN +ve. |
* MIB-1: usu 1-3%. |
====Molecular==== |
* Distinct methylation profile. |
* Recurent losses on 2p and Chr. 14.<ref>{{Cite journal | last1 = Capper | first1 = D. | last2 = Stichel | first2 = D. | last3 = Sahm | first3 = F. | last4 = Jones | first4 = DTW. | last5 = Schrimpf | first5 = D. | last6 = Sill | first6 = M. | last7 = Schmid | first7 = S. | last8 = Hovestadt | first8 = V. | last9 = Re... |
<gallery> |
File:Cerebellar liponeurocytoma.jpg | Liponeurocytoma, HE (WC/Marvin101). |
File:Liponeurocytoma Synaptophysin.jpg | Liponeurocytoma, Synapto (WC/Marvin101). |
File:Cerebellar Liponeurocytoma HE.jpg | Liponeurocytoma, HE (WC/jensflorian). |
File:Cerebellar Liponeurocytoma Synaptophysin.jpg | Liponeurocytoma, Synapto (WC/jensflorian). |
</gallery> |
====DDx==== |
* [[Medulloblastoma]] |
* [[Neurocytoma]] |
===Gangliocytoma=== |
* Grade I WHO neuronal tumour. |
** ICD-O code: 9492/0 |
* Groups of irregular large neurons. |
* Non-neoplastic, reticulin-rich glial stroma. |
===Ganglioglioma=== |
:'''Not''' to be confused with ''[[ganglioneuroma]]''. |
====General==== |
*Gangliolioma: Grade I WHO mixed neuronal-glial tumour (ICD-O code: 9505/1). |
*Anaplastic ganglioglioma: Grade III (ICD-O: 9505/3) |
*Rare (approx. 0.5% of all CNS tumors). |
*Usu. temporal lobe. |
*Predominantly children (mean age: 9 years). |
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