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→Diffuse midline glioma, H3 K27M mutant: update
Jensflorian (talk | contribs) (→Diffuse midline glioma, H3 K27M mutant: update) |
Jensflorian (talk | contribs) (→Diffuse midline glioma, H3 K27M mutant: update) |
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* Newly defined entity since WHO 2016 classification.<ref>{{Cite journal | last1 = Louis | first1 = DN. | last2 = Perry | first2 = A. | last3 = Reifenberger | first3 = G. | last4 = von Deimling | first4 = A. | last5 = Figarella-Branger | first5 = D. | last6 = Cavenee | first6 = WK. | last7 = Ohgaki | first7 = H. | last8 = Wiestler | first8 = OD. | last9 = Kleihues | first9 = P. | title = The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. | journal = Acta Neuropathol | volume = 131 | issue = 6 | pages = 803-20 | month = Jun | year = 2016 | doi = 10.1007/s00401-016-1545-1 | PMID = 27157931 }}</ref> | * Newly defined entity since WHO 2016 classification.<ref>{{Cite journal | last1 = Louis | first1 = DN. | last2 = Perry | first2 = A. | last3 = Reifenberger | first3 = G. | last4 = von Deimling | first4 = A. | last5 = Figarella-Branger | first5 = D. | last6 = Cavenee | first6 = WK. | last7 = Ohgaki | first7 = H. | last8 = Wiestler | first8 = OD. | last9 = Kleihues | first9 = P. | title = The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. | journal = Acta Neuropathol | volume = 131 | issue = 6 | pages = 803-20 | month = Jun | year = 2016 | doi = 10.1007/s00401-016-1545-1 | PMID = 27157931 }}</ref> | ||
* Distinct biological and clinical group with poor prognosis.<ref>{{Cite journal | last1 = Khuong-Quang | first1 = DA. | last2 = Buczkowicz | first2 = P. | last3 = Rakopoulos | first3 = P. | last4 = Liu | first4 = XY. | last5 = Fontebasso | first5 = AM. | last6 = Bouffet | first6 = E. | last7 = Bartels | first7 = U. | last8 = Albrecht | first8 = S. | last9 = Schwartzentruber | first9 = J. | title = K27M mutation in histone H3.3 defines clinically and biologically distinct subgroups of pediatric diffuse intrinsic pontine gliomas. | journal = Acta Neuropathol | volume = 124 | issue = 3 | pages = 439-47 | month = Sep | year = 2012 | doi = 10.1007/s00401-012-0998-0 | PMID = 22661320 }}</ref> | * Distinct biological and clinical group with poor prognosis.<ref>{{Cite journal | last1 = Khuong-Quang | first1 = DA. | last2 = Buczkowicz | first2 = P. | last3 = Rakopoulos | first3 = P. | last4 = Liu | first4 = XY. | last5 = Fontebasso | first5 = AM. | last6 = Bouffet | first6 = E. | last7 = Bartels | first7 = U. | last8 = Albrecht | first8 = S. | last9 = Schwartzentruber | first9 = J. | title = K27M mutation in histone H3.3 defines clinically and biologically distinct subgroups of pediatric diffuse intrinsic pontine gliomas. | journal = Acta Neuropathol | volume = 124 | issue = 3 | pages = 439-47 | month = Sep | year = 2012 | doi = 10.1007/s00401-012-0998-0 | PMID = 22661320 }}</ref> | ||
** EGFR amplification is usu. absent.<ref>{{Cite journal | last1 = Meyronet | first1 = D. | last2 = Esteban-Mader | first2 = M. | last3 = Bonnet | first3 = C. | last4 = Joly | first4 = MO. | last5 = Uro-Coste | first5 = E. | last6 = Amiel-Benouaich | first6 = A. | last7 = Forest | first7 = F. | last8 = Rousselot-Denis | first8 = C. | last9 = Burel-Vandenbos | first9 = F. | title = Characteristics of H3 K27M-mutant gliomas in adults. | journal = Neuro Oncol | volume = 19 | issue = 8 | pages = 1127-1134 | month = Aug | year = 2017 | doi = 10.1093/neuonc/now274 | PMID = 28201752 }}</ref> | |||
** Tumors usu. have unmethylated MGMT promotor.<ref>{{Cite journal | last1 = Banan | first1 = R. | last2 = Christians | first2 = A. | last3 = Bartels | first3 = S. | last4 = Lehmann | first4 = U. | last5 = Hartmann | first5 = C. | title = Absence of MGMT promoter methylation in diffuse midline glioma, H3 K27M-mutant. | journal = Acta Neuropathol Commun | volume = 5 | issue = 1 | pages = 98 | month = 12 | year = 2017 | doi = 10.1186/s40478-017-0500-2 | PMID = 29246238 }}</ref> | |||
* MRI: May be or be not enhancing. | * MRI: May be or be not enhancing. | ||
*Histologic spectrum ranges from minimal hypercellularity to full-blown glioblastoma. | *Histologic spectrum ranges from minimal hypercellularity to full-blown glioblastoma. | ||