Difference between revisions of "Diffuse leptomeningeal glioneuronal tumour"

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==Molecular==
==Molecular==
* Mixture of partially oligodendroglioma, partially pilocytic astrocytoma features.<ref name="pmid29766299">{{cite journal |vauthors=Deng MY, Sill M, Chiang J, Schittenhelm J, Ebinger M, Schuhmann MU, Monoranu CM, Milde T, Wittmann A, Hartmann C, Sommer C, Paulus W, Gärtner J, Brück W, Rüdiger T, Leipold A, Jaunmuktane Z, Brandner S, Giangaspero F, Nozza P, Mora J, Morales la Madrid A, Cruz Martinez O, Hansford JR, Pietsch T, Tietze A, Hernáiz-Driever P, Stoler I, Capper D, Korshunov A, Ellison DW, von Deimling A, Pfister SM, Sahm F, Jones DTW |title=Molecularly defined diffuse leptomeningeal glioneuronal tumor (DLGNT) comprises two subgroups with distinct clinical and genetic features |journal=Acta Neuropathol |volume=136 |issue=2 |pages=239–253 |date=August 2018 |pmid=29766299 |doi=10.1007/s00401-018-1865-4 |url=}}</ref>
* 1p loss  in 100% (or combined 1p/19q loss in 15-50%)<ref>{{Cite journal  | last1 = Bourne | first1 = TD. | last2 = Mandell | first2 = JW. | last3 = Matsumoto | first3 = JA. | last4 = Jane | first4 = JA. | last5 = Lopes | first5 = MB. | title = Primary disseminated leptomeningeal oligodendroglioma with 1p deletion. Case report. | journal = J Neurosurg | volume = 105 | issue = 6 Suppl | pages = 465-9 | month = Dec | year = 2006 | doi = 10.3171/ped.2006.105.6.465 | PMID = 17184079 }}</ref>
* 1p loss  in 100% (or combined 1p/19q loss in 15-50%)<ref>{{Cite journal  | last1 = Bourne | first1 = TD. | last2 = Mandell | first2 = JW. | last3 = Matsumoto | first3 = JA. | last4 = Jane | first4 = JA. | last5 = Lopes | first5 = MB. | title = Primary disseminated leptomeningeal oligodendroglioma with 1p deletion. Case report. | journal = J Neurosurg | volume = 105 | issue = 6 Suppl | pages = 465-9 | month = Dec | year = 2006 | doi = 10.3171/ped.2006.105.6.465 | PMID = 17184079 }}</ref>
* 1p gain (35-100% depending on subtype).
** 1p loss is usu. absent in [[pilocytic astrocytoma]]
** 1p loss is usu. absent in [[pilocytic astrocytoma]]
**Cases without 1p/19q loss reported.<ref>{{Cite journal  | last1 = Preuss | first1 = M. | last2 = Christiansen | first2 = H. | last3 = Merkenschlager | first3 = A. | last4 = Hirsch | first4 = FW. | last5 = Kiess | first5 = W. | last6 = Müller | first6 = W. | last7 = Kästner | first7 = S. | last8 = Henssler | first8 = A. | last9 = Pekrun | first9 = A. | title = Disseminated oligodendroglial-like leptomeningeal tumors: preliminary diagnostic and therapeutic results for a novel tumor entity [corrected]. | journal = J Neurooncol | volume = 124 | issue = 1 | pages = 65-74 | month = Aug | year = 2015 | doi = 10.1007/s11060-015-1735-z | PMID = 25672644 }}</ref>
**Cases without 1p/19q loss reported.<ref>{{Cite journal  | last1 = Preuss | first1 = M. | last2 = Christiansen | first2 = H. | last3 = Merkenschlager | first3 = A. | last4 = Hirsch | first4 = FW. | last5 = Kiess | first5 = W. | last6 = Müller | first6 = W. | last7 = Kästner | first7 = S. | last8 = Henssler | first8 = A. | last9 = Pekrun | first9 = A. | title = Disseminated oligodendroglial-like leptomeningeal tumors: preliminary diagnostic and therapeutic results for a novel tumor entity [corrected]. | journal = J Neurooncol | volume = 124 | issue = 1 | pages = 65-74 | month = Aug | year = 2015 | doi = 10.1007/s11060-015-1735-z | PMID = 25672644 }}</ref>
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** BRAF-KIAA1549 fusions are usu. absent in [[oligodendroglioma]].<ref>{{Cite journal  | last1 = Gierke | first1 = M. | last2 = Sperveslage | first2 = J. | last3 = Schwab | first3 = D. | last4 = Beschorner | first4 = R. | last5 = Ebinger | first5 = M. | last6 = Schuhmann | first6 = MU. | last7 = Schittenhelm | first7 = J. | title = Analysis of IDH1-R132 mutation, BRAF V600 mutation and KIAA1549-BRAF fusion transcript status in central nervous system tumors supports pediatric tumor classification. | journal = J Cancer Res Clin Oncol | volume =  | issue =  | pages =  | month = Jun | year = 2015 | doi = 10.1007/s00432-015-2006-2 | PMID = 26115961 }}</ref>
** BRAF-KIAA1549 fusions are usu. absent in [[oligodendroglioma]].<ref>{{Cite journal  | last1 = Gierke | first1 = M. | last2 = Sperveslage | first2 = J. | last3 = Schwab | first3 = D. | last4 = Beschorner | first4 = R. | last5 = Ebinger | first5 = M. | last6 = Schuhmann | first6 = MU. | last7 = Schittenhelm | first7 = J. | title = Analysis of IDH1-R132 mutation, BRAF V600 mutation and KIAA1549-BRAF fusion transcript status in central nervous system tumors supports pediatric tumor classification. | journal = J Cancer Res Clin Oncol | volume =  | issue =  | pages =  | month = Jun | year = 2015 | doi = 10.1007/s00432-015-2006-2 | PMID = 26115961 }}</ref>
*BRAF V600E-negative.
*BRAF V600E-negative.
* IDH1/2-Wildtype.
* Two methylation class subgroups (DLGNT MC-1 and MC-2)
** 1p gain in all MC-2 class cases.
** MC-1 class cases are younger and have unfavourable prognosis.


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