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→IHC: moved from neuropathology tumours
Jensflorian (talk | contribs) (→Molecular: pediatric GBM) |
Jensflorian (talk | contribs) (→IHC: moved from neuropathology tumours) |
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*Median survival is measured in months.<ref>{{Cite journal | last1 = Jubelirer | first1 = SJ. | title = A review of the treatment and survival rates of 138 patients with glioblastoma multiforme. | journal = W V Med J | volume = 92 | issue = 4 | pages = 186-90 | month = | year = | doi = | PMID = 8772403 }}</ref> | *Median survival is measured in months.<ref>{{Cite journal | last1 = Jubelirer | first1 = SJ. | title = A review of the treatment and survival rates of 138 patients with glioblastoma multiforme. | journal = W V Med J | volume = 92 | issue = 4 | pages = 186-90 | month = | year = | doi = | PMID = 8772403 }}</ref> | ||
*Only about 5% can expect to survive more than three years.<ref name=pmid17785346>{{Cite journal | last1 = Krex | first1 = D. | last2 = Klink | first2 = B. | last3 = Hartmann | first3 = C. | last4 = von Deimling | first4 = A. | last5 = Pietsch | first5 = T. | last6 = Simon | first6 = M. | last7 = Sabel | first7 = M. | last8 = Steinbach | first8 = JP. | last9 = Heese | first9 = O. | title = Long-term survival with glioblastoma multiforme. | journal = Brain | volume = 130 | issue = Pt 10 | pages = 2596-606 | month = Oct | year = 2007 | doi = 10.1093/brain/awm204 | PMID = 17785346 }}</ref> | *Only about 5% can expect to survive more than three years.<ref name=pmid17785346>{{Cite journal | last1 = Krex | first1 = D. | last2 = Klink | first2 = B. | last3 = Hartmann | first3 = C. | last4 = von Deimling | first4 = A. | last5 = Pietsch | first5 = T. | last6 = Simon | first6 = M. | last7 = Sabel | first7 = M. | last8 = Steinbach | first8 = JP. | last9 = Heese | first9 = O. | title = Long-term survival with glioblastoma multiforme. | journal = Brain | volume = 130 | issue = Pt 10 | pages = 2596-606 | month = Oct | year = 2007 | doi = 10.1093/brain/awm204 | PMID = 17785346 }}</ref> | ||
*Current classification recognizes three types: | |||
** Glioblastoma, IDH-wildtype (aka primary GBM, ICD-O: 9440/3). | |||
** Glioblastoma, IDH-mutant (aka secondary GBM, ICD-O: 9445/3). | |||
** Glioblastoma, NOS (lack of molecular data). | |||
==Macroscopy== | ==Macroscopy== | ||
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File:GBM layers.jpg | Resection borders in a recurrent GBM (WC/jensflorian) | File:GBM layers.jpg | Resection borders in a recurrent GBM (WC/jensflorian) | ||
File:Glioblastoma-radiation_changes_HE.jpg | Radiation changes in a recurrent GBM (WC/jensflorian) | File:Glioblastoma-radiation_changes_HE.jpg | Radiation changes in a recurrent GBM (WC/jensflorian) | ||
File:GBM_nested_epithelial.jpg | Nested epitheloid appearance in a GBM specimen (WC/jensflorian) | |||
Image:Glioblastoma_%281%29.jpg | Glioblastoma - pseudopalisading of tumour cells (WC) | |||
Image:Glioblastoma_-_high_mag.jpg | Glioblastoma with fragment of near-normal white matter - high mag. (WC) | |||
Image:Anaplastic_astrocytoma_-_very_high_mag_-_cropped.jpg | Anaplastic astrocytoma - very high mag. (WC) | |||
</gallery> | </gallery> | ||
www: | www: | ||
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*[[MAP2]] +ve. | *[[MAP2]] +ve. | ||
*[[IDH-1]] -ve (95%). | *[[IDH-1]] -ve (95%). | ||
**+ve if developed from lower grade astrocytoma (secondary GBM). | **+ve if developed from lower grade astrocytoma (secondary GBM) -> classify tumor as Glioblastoma, IDH-mutant. | ||
*[[WT-1]] +ve (cytoplasm). | *[[WT-1]] +ve (cytoplasm). | ||
*p53 +ve (70%). | *p53 +ve (70%). | ||
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*panCK -ve (except for GBM with epithelial component). | *panCK -ve (except for GBM with epithelial component). | ||
*[[ATRX]]: +ve (no loss, nuclei) | *[[ATRX]]: +ve (no loss, nuclei) | ||
**-ve if developed from lower grade astrocytoma (secondary GBM). | **-ve if developed from lower grade astrocytoma (secondary GBM). | ||
*EMA: Dot-like expression less common than in [[ependymoma]]s. | *EMA: Dot-like expression less common than in [[ependymoma]]s. | ||
*MIB-1 usu. 15-30%, but varies greatly. | *MIB-1 usu. 15-30%, but varies greatly. | ||
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File:Wilms tumor protein wt1 immunohistocehmistry glioblastoma.JPG | WT1 immunostaining in GBM (WC/jensflorian) | File:Wilms tumor protein wt1 immunohistocehmistry glioblastoma.JPG | WT1 immunostaining in GBM (WC/jensflorian) | ||
File:Glioblastoma P53.jpg | GBM with strong p53 immunreactivity (WC/jensflorian) | File:Glioblastoma P53.jpg | GBM with strong p53 immunreactivity (WC/jensflorian) | ||
File:Glioblastoma Ck7.jpg|CK7 staining in glioblastoma with epithelial component (WC/jensflorian) | File:Glioblastoma Ck7.jpg|CK7 staining in glioblastoma with epithelial component (WC/jensflorian) | ||
File:GBM_with_Neuropil_island_MIB1.jpg|Reduced proliferation in neuropil-like islands (MIB1). | File:GBM_with_Neuropil_island_MIB1.jpg|Reduced proliferation in neuropil-like islands (MIB1). | ||
File:GBM_with_Neuropil_island_Syn.jpg |Synaptophysin immunoreactivity in neuropil-like islands (WC/jensflorian). | File:GBM_with_Neuropil_island_Syn.jpg |Synaptophysin immunoreactivity in neuropil-like islands (WC/jensflorian). | ||
Image:Anaplastic_astrocytoma_-_gfap_-_very_high_mag.jpg | Anaplastic astrocytic tumor cells - GFAP - very high mag. (WC) | |||
</gallery> | </gallery> | ||
==Molecular== | ==Molecular== | ||
*IDH1/2 sequencing in cases below 55 years is mandatory to separate between Glioblastoma, IDH-wildtype and Glioblastoma IDH-mutant. | |||
** In cases above 55 years, negative IDH1 R132H immunohistochemistry may be sufficient. | |||
* 70% of IDH-wildtype glioblastoma show chr.7 gain and chr.10 loss.<ref>{{Cite journal | last1 = Ceccarelli | first1 = M. | last2 = Barthel | first2 = FP. | last3 = Malta | first3 = TM. | last4 = Sabedot | first4 = TS. | last5 = Salama | first5 = SR. | last6 = Murray | first6 = BA. | last7 = Morozova | first7 = O. | last8 = Newton | first8 = Y. | last9 = Radenbaugh | first9 = A. | title = Molecular Profiling Reveals Biologically Discrete Subsets and Pathways of Progression in Diffuse Glioma. | journal = Cell | volume = 164 | issue = 3 | pages = 550-63 | month = Jan | year = 2016 | doi = 10.1016/j.cell.2015.12.028 | PMID = 26824661 }}</ref> | |||
*Seen in inherited tumor syndromes: | *Seen in inherited tumor syndromes: | ||
**Lynch | **[[Lynch syndrome]] | ||
**[[Neurofibromatosis 1]] | **[[Neurofibromatosis 1]] | ||
**Li-Fraumeni | **[[Li-Fraumeni syndrome]] | ||
**Turcot-Syndrome | **Turcot-Syndrome | ||
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*Diagnostic/therapeutic relevant markers: | *Diagnostic/therapeutic relevant markers: | ||
**MGMT promoter methylation status | **[[MGMT]] promoter methylation status<ref>{{Cite journal | last1 = Quillien | first1 = V. | last2 = Lavenu | first2 = A. | last3 = Karayan-Tapon | first3 = L. | last4 = Carpentier | first4 = C. | last5 = Labussière | first5 = M. | last6 = Lesimple | first6 = T. | last7 = Chinot | first7 = O. | last8 = Wager | first8 = M. | last9 = Honnorat | first9 = J. | title = Comparative assessment of 5 methods (methylation-specific polymerase chain reaction, MethyLight, pyrosequencing, methylation-sensitive high-resolution melting, and immunohistochemistry) to analyze O6-methylguanine-DNA-methyltranferase in a series of 100 glioblastoma patients. | journal = Cancer | volume = 118 | issue = 17 | pages = 4201-11 | month = Sep | year = 2012 | doi = 10.1002/cncr.27392 | PMID = 22294349 }}</ref> | ||
**Absence of LOH 1p/19q (otherwise classify tumor as [[oligodendroglioma]]). | **Absence of LOH 1p/19q (otherwise classify tumor as [[oligodendroglioma]]).<ref>{{Cite journal | last1 = Masui | first1 = K. | last2 = Mischel | first2 = PS. | last3 = Reifenberger | first3 = G. | title = Molecular classification of gliomas. | journal = Handb Clin Neurol | volume = 134 | issue = | pages = 97-120 | month = | year = 2016 | doi = 10.1016/B978-0-12-802997-8.00006-2 | PMID = 26948350 }}</ref> | ||
==See also== | ==See also== | ||