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Difference between revisions of "Diffuse leptomeningeal glioneuronal tumour"
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Diffuse leptomeningeal glioneuronal tumour (view source)
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==General== | ==General== | ||
Features: | Features: | ||
*Currently no WHO grade assigned. | *Currently no WHO grade assigned. Molecular class MC-1 corresponds to WHO I, class MC-2 to WHO II/III<ref name="pmid29766299">{{cite journal |authors=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> | ||
*Low-grade lesion but anaplastic progression reported. | |||
* Previously known as '''Disseminated oligodendroglial-like leptomeningeal tumour''' (abbreviated DOLN) | * Previously known as '''Disseminated oligodendroglial-like leptomeningeal tumour''' (abbreviated DOLN) | ||
* | ** AKA "disseminated oligodendroglial-like leptomeningeal tumor of childhood" (DOGLT).<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> | ||
*AKA | |||
*Intermediate prognosis. | *Intermediate prognosis. | ||
==Gross== | ==Gross== | ||
Features: | Features: | ||
* | *Multiple tumor sites (60-85% spinal). | ||
*Diffuse leptomeningeal expansion. | *Diffuse leptomeningeal expansion. | ||
**Hydrocaphalus due to CSF occlusion. | **Hydrocaphalus due to CSF occlusion. | ||
| Line 39: | Line 38: | ||
==Molecular== | ==Molecular== | ||
* 1p loss (or 1p/19q loss)<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> | * Mixture of partially oligodendroglioma, partially pilocytic astrocytoma features.<ref name="pmid29766299">{{cite journal |authors=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 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 a more favourable prognosis. | |||
==Images== | ==Images== | ||