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Anaplastic ganglioglioma: |
*Brisk mitotic activity |
*Necrosis |
====IHC==== |
*Neurons: |
**[[MAP2]] +ve |
**Synaptophysin +ve |
***Perikaryal surface immunoreactivity for synaptophysin.<ref name="pmid8246055">{{cite journal |authors=Lang FF, Epstein FJ, Ransohoff J, Allen JC, Wisoff J, Abbott IR, Miller DC |title=Central nervous system gangliogliomas. Part 2: Clinical outcome |journal=J. Neurosurg. |volume=79 |issue=6 |pages=867–73 |date=Decemb... |
***This is however also seen in reactive changes.<ref name="pmid9591724">{{cite journal |authors=Quinn B |title=Synaptophysin staining in normal brain: importance for diagnosis of ganglioglioma |journal=Am. J. Surg. Pathol. |volume=22 |issue=5 |pages=550–6 |date=May 1998 |pmid=9591724 |doi=10.1097/00000478-199805000-00... |
** Neurofilament +ve |
** Chromogranin +ve |
*Glia: |
**CD34+/-ve |
*BRAF V600E +ve (approx. 25%, mainly ganglion cells). |
*MAP2: usu. absent. |
*MIB-1 (low, but resembles proliferative tumor component). |
====Molecular==== |
*BRAF V600E-mutated(approx. 25%).<ref>{{Cite journal | last1 = Schindler | first1 = G. | last2 = Capper | first2 = D. | last3 = Meyer | first3 = J. | last4 = Janzarik | first4 = W. | last5 = Omran | first5 = H. | last6 = Herold-Mende | first6 = C. | last7 = Schmieder | first7 = K. | last8 = Wesseling | first8 = P. | l... |
**BRAF V600E antibody stains especially neuronal cells.<ref>{{Cite journal | last1 = Koelsche | first1 = C. | last2 = Wöhrer | first2 = A. | last3 = Jeibmann | first3 = A. | last4 = Schittenhelm | first4 = J. | last5 = Schindler | first5 = G. | last6 = Preusser | first6 = M. | last7 = Lasitschka | first7 = F. | last8 ... |
*IDH1/2 wt. |
*No 1p/19q codeletion. |
*Usu. Chr. 7 gain. |
*Rare cases with KIAA1459-BRAF fusion.<ref>{{Cite journal | last1 = Pekmezci | first1 = M. | last2 = Villanueva-Meyer | first2 = JE. | last3 = Goode | first3 = B. | last4 = Van Ziffle | first4 = J. | last5 = Onodera | first5 = C. | last6 = Grenert | first6 = JP. | last7 = Bastian | first7 = BC. | last8 = Chamyan | fir... |
** DDx: consider pilocytic astrocytoma with ganglioglioma component. <ref>{{Cite journal | last1 = Mesturoux | first1 = L. | last2 = Durand | first2 = K. | last3 = Pommepuy | first3 = I. | last4 = Robert | first4 = S. | last5 = Caire | first5 = F. | last6 = Labrousse | first6 = F. | title = Molecular Analysis of Tumor... |
*CDKN2A deletions, ATRX loss and TERT promotor mutations in anaplastic ganglioglioma.<ref>{{Cite journal | last1 = Zanello | first1 = M. | last2 = Pages | first2 = M. | last3 = Tauziède-Espariat | first3 = A. | last4 = Saffroy | first4 = R. | last5 = Puget | first5 = S. | last6 = Lacroix | first6 = L. | last7 = Dezami... |
**DD: consider anaplastic astrocytoma with piloid features (similiar molecular profile).<ref>{{Cite journal | last1 = Reinhardt | first1 = A. | last2 = Stichel | first2 = D. | last3 = Schrimpf | first3 = D. | last4 = Sahm | first4 = F. | last5 = Korshunov | first5 = A. | last6 = Reuss | first6 = DE. | last7 = Koelsche... |
</ref> |
*H3F3A wildtype. |
**DDx: consider [[Diffuse hemispheric glioma, H3 G34-mutant]] (rare cases may contain dysplastic ganglion cells).<ref>{{Cite journal | last1 = Andreiuolo | first1 = F. | last2 = Lisner | first2 = T. | last3 = Zlocha | first3 = J. | last4 = Kramm | first4 = C. | last5 = Koch | first5 = A. | last6 = Bison | first6 = B. ... |
*Rare cases with co-occurrence of K27M mutation.<ref>{{Cite journal | last1 = Pagès | first1 = M. | last2 = Beccaria | first2 = K. | last3 = Boddaert | first3 = N. | last4 = Saffroy | first4 = R. | last5 = Besnard | first5 = A. | last6 = Castel | first6 = D. | last7 = Fina | first7 = F. | last8 = Barets | first8 = D. ... |
===Images=== |
<gallery> |
File:Ganglioglioma lymphocytic cuffing PAS.jpg | Lymphocytic cuffing in ganglioglioma (WC/jensflorian) |
File:Ganglioglioma calcification.jpg | Calcification in ganglioglioma (WC/jensflorian) |
File:Ganglioglioma Cd34 x200.jpg | CD34 immunostain in ganglioglioma (WC/jensflorian) |
File:Anaplastic ganglioglioma HE.jpg | Pleomorphic ganglion cells in ganglioglioma (WC/jensflorian) |
</gallery> |
==Prognosis== |
*Very good (10-year OS: 97%) |
*Primary treatment: surgery. |
**Seizure free outcome: 81%. |
**Incomplete resection as major factor for persisting epilepsia.<ref>{{Cite journal | last1 = Devaux | first1 = B. | last2 = Chassoux | first2 = F. | last3 = Landré | first3 = E. | last4 = Turak | first4 = B. | last5 = Laurent | first5 = A. | last6 = Zanello | first6 = M. | last7 = Mellerio | first7 = C. | last8 = Var... |
====DDx:==== |
*[[DNT]]. |
*[[Oligodendroglioma]]. |
*[[PXA]]. |
*Desmoplastic infantile astrocytoma and ganglioglioma. |
*Cortical tuber. |
*Trapped cortical neurons in diffuse astrocytoma. |
*Papillary glioneuronal tumor. |
==See also== |
*[[Neuropathology tumours]]. |
*[[Pilocytic Astrocytoma]]. |
==References== |
{{Reflist|2}} |
[[Category:Diagnosis]] |
[[Category:Neuropathology tumours]] |
aaa1917ffeb938e4edaed76e3b29b82920e3c674292bfa395149dc71054c8882 librepath-ganglioglioma.txt |
{{ Infobox diagnosis |
| Name = {{PAGENAME}} |
| Image = Hemangioblastoma Histology HE.jpg |
| Width = |
| Caption = Cerebellar hemangioblastoma. |
| Synonyms = |
| Micro = vascular tumour with large polygonal stromal cells with hyperchromatic nuclei and vacuolar cytoplasm |
| Subtypes = |
| LMDDx = metastatic [[clear cell renal cell carcinoma]] |
| Stains = |
| IHC = alpha-inhibin +ve, NSE +ve, EMA -ve |
| EM = |
| Molecular = |
| IF = |
| Gross = |
| Grossing = |
| Site = brain - usu. [[cerebellum]] |
| Assdx = |
| Syndromes = [[von Hippel-Lindau disease]] |
| Clinicalhx = |
| Signs = |
| Symptoms = |
| Prevalence = |
| Bloodwork = |
| Rads = |
| Endoscopy = |
| Prognosis = good (WHO grade I) |
| Other = |
| ClinDDx = |
| Tx = |
}} |
'''Hemangioblastoma''' is a low grade [[brain tumour]] tumour typically found the [[cerebellum]]. |
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