Difference between revisions of "Oligodendroglioma"

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Oligodendroglioma (view source)

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| Image      = Oligodendroglioma1_high_mag.jpg
| Image      = Oligodendroglioma1_high_mag.jpg
| Width      =
| Width      =
| Caption    = Oligodendroglioma. [[H&E stain]].
| Caption    = Oligodendroglioma,IDH mutant and 1p/19q codeleted. [[H&E stain]].
| Synonyms  =
| Synonyms  =
| Micro      = highly cellular lesion composed of cells resembling ''fried eggs'' (oligodendrocytes) with a round nucleus (important), distinct cell borders, +/-clear cytoplasm - useful feature (if present), acutely branched capillary sized vessels ("chicken-wire" like appearance), calcifications
| Micro      = highly cellular lesion composed of cells resembling ''fried eggs'' (oligodendrocytes) with a round nucleus (important), distinct cell borders, +/-clear cytoplasm - useful feature (if present), acutely branched capillary sized vessels ("chicken-wire" like appearance), calcifications
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| IHC        =
| IHC        =
| EM        =
| EM        =
| Molecular  =
| Molecular  = +/-loss of 1p and 19q (common)
| IF        =
| IF        =
| Gross      =
| Gross      =
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| Prevalence =
| Prevalence =
| Bloodwork  =
| Bloodwork  =
| Rads      =
| Rads      = intra-axial mass, +/-calcifications (best seen on CT), nonenhancing or enhancing
| Endoscopy  =
| Endoscopy  =
| Prognosis  = moderate - dependent on grade
| Prognosis  = moderate - dependent on grade
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| Tx        =
| Tx        =
}}
}}
'''Oligodendroglioma''' is [[CNS tumour]] that is typically in the cerebral hemispheres.  
'''Oligodendroglioma, IDH mutant and 1p/19q codeleted''' is [[CNS tumour]] that is typically in the cerebral hemispheres. Molecular analysis of IDH1/2 hotspots and LOH1p/19q testing is required for diagnosis.


==General==
==General==
*Do ''not'' arise from oligodendrocytes.
*Do ''not'' arise from [[oligodendrocyte]]s, although tumor cells look very similiar.<ref>{{Cite journal  | last1 = Hartmann | first1 = C. | last2 = von Deimling | first2 = A. | title = Molecular pathology of oligodendroglial tumors. | journal = Recent Results Cancer Res | volume = 171 | issue =  | pages = 25-49 | month =  | year = 2009 | doi = 10.1007/978-3-540-31206-2_2 | PMID = 19322536 }}</ref>
**Arise from ''glial precursor cells''.
**Arise from ''glial precursor cells''.


Usual location:
Prognosis by flavours (average survival):<ref name=Ref_PSNP98>{{Ref PSNP|98}}</ref>
*Cerebral hemispheres- most often frontal lobe.  
*WHO CNS grade 2: 10-15 years.
*Posterior fossa (rare)
*WHO CNS grade 3: 3-5 years.
 
Oligodendrogliomas account for approx 5% of all [[glioma]]s.
* Numbers may be higher when stringent classifiation criteria are not applied.
* Peak incidence: 40-45 years.
* First description of the tumor in 1926.
 
The fifth edition of WHO classification recognizes two tumor subtypes:
*Oligodendroglioma, IDH-mutant and 1p/19q-codeleted, WHO CNS Grade 2 (ICD-O: 9450/3).
*Oligodendroglioma, IDH-mutant and 1p/19q-codeleted, WHO CNS Grade 3 (ICD-O: 9451/3).
 
The terminology anaplastic oligodendroglioma or oligoastrocytoma is depreceated. <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>
 
==Gross/radiologic==
Location:
*Cerebral hemispheres - most often (50-60%) frontal lobe, followed by parietal and temporal lobes.<ref name=Ref_PSNP94>{{Ref PSNP|94}}</ref>
*Posterior fossa (rare).
*Intramedullary spinal cord (very rare).
*Intramedullary spinal cord (very rare).


Prognosis by flavours (average survival):<ref name=Ref_PSNP98>{{Ref PSNP|98}}</ref>
Radiologic features:<ref name=Ref_PSNP94>{{Ref PSNP|94}}</ref>  
*WHO grade II: 10-15 years.
*Intra-axial mass.
*WHO grade III: 3-5 years.
*+/-Calcifications (best seen on CT).
*Nonenhancing or enhancing.
*Occasionally well-circumscribed.
*Widespread dissemination in a gliomatosis cerebri fashion is very rare (DDx: Diffuse leptomeningeal glioneuronal tumour).
 
Clinical symptoms:
*Seizures (2/3 of all cases).
*Intracranial pressure.
*Focal neurologic decifits.
*Cognitive changes.
*Incidental finding in 10%.


==Microscopic==
==Microscopic==
Features:
Features:
*Diffusely growing tumor.
*Highly cellular lesion composed of:
*Highly cellular lesion composed of:
**Cells resembling ''fried eggs'' (oligodendrocytes) with:
**Cells resembling ''fried eggs'' (oligodendrocytes) with:
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***Abundant, delicate appearing; may vaguely resemble a paraganglioma at low power.
***Abundant, delicate appearing; may vaguely resemble a paraganglioma at low power.
*Calcifications - important feature.<ref>URL: [http://www.emedicine.com/radio/topic481.htm http://www.emedicine.com/radio/topic481.htm].</ref>
*Calcifications - important feature.<ref>URL: [http://www.emedicine.com/radio/topic481.htm http://www.emedicine.com/radio/topic481.htm].</ref>
*Perifocal edema uncommon.
*Few tumors may exhibit [[eosinophilic granular body|eosinophilic granular bodies]].
*Some tumors may show a [[Polar Spongioblastoma|Spongioblastoma]]-like growth pattern.
Anaplastic (grade 3) criteria:<ref>{{Cite journal  | last1 = Giannini | first1 = C. | last2 = Scheithauer | first2 = BW. | last3 = Weaver | first3 = AL. | last4 = Burger | first4 = PC. | last5 = Kros | first5 = JM. | last6 = Mork | first6 = S. | last7 = Graeber | first7 = MB. | last8 = Bauserman | first8 = S. | last9 = Buckner | first9 = JC. | title = Oligodendrogliomas: reproducibility and prognostic value of histologic diagnosis and grading. | journal = J Neuropathol Exp Neurol | volume = 60 | issue = 3 | pages = 248-62 | month = Mar | year = 2001 | doi =  | PMID = 11245209 }}</ref>
*"Significant" or "brisk" mitotic activity.
**That means for most neuropathologists >= 6 mitoses per 10 HPF.
*Microvacular proliferation.
*Necrosis.
*Rarely multnucleated cells.


Note:
Note:
*Tumour cells may be plasmacytoid, i.e. have a [[plasma cell]]-like appearance.<ref name=pmid17284109>{{Cite journal  | last1 = Aldape | first1 = K. | last2 = Burger | first2 = PC. | last3 = Perry | first3 = A. | title = Clinicopathologic aspects of 1p/19q loss and the diagnosis of oligodendroglioma. | journal = Arch Pathol Lab Med | volume = 131 | issue = 2 | pages = 242-51 | month = Feb | year = 2007 | doi = 10.1043/1543-2165(2007)131[242:CAOQLA]2.0.CO;2 | PMID = 17284109 | URL = http://www.archivesofpathology.org/doi/full/10.1043/1543-2165(2007)131%5B242:CAOQLA%5D2.0.CO;2 }}</ref>
* The clear cytoplasm around the nucleus is a fixation artefact and not seen in smear, frozen sections or rapid fixation.
 
* Tumour cells may be plasmacytoid, i.e. have a [[plasma cell]]-like appearance.<ref name=pmid17284109>{{Cite journal  | last1 = Aldape | first1 = K. | last2 = Burger | first2 = PC. | last3 = Perry | first3 = A. | title = Clinicopathologic aspects of 1p/19q loss and the diagnosis of oligodendroglioma. | journal = Arch Pathol Lab Med | volume = 131 | issue = 2 | pages = 242-51 | month = Feb | year = 2007 | doi = 10.1043/1543-2165(2007)131[242:CAOQLA]2.0.CO;2 | PMID = 17284109 | URL = http://www.archivesofpathology.org/doi/full/10.1043/1543-2165(2007)131%5B242:CAOQLA%5D2.0.CO;2 }}</ref>
DDx:
**Also called minigemistocytes.
*[[Neurocytoma]] also have perinuclear clearing and well-defined cellular borders.
**The are usually strong GFAP+ve.
**Pineocytomatous/neurocytic rosettes = (irregular) rosette with a large meshwork of fibers (neuropil) at the centre.
*Few neural tumours have round nuclei - DDx in intraoperative specimen:
 
Notes:
*Few neural tumours have round nuclei - DDx:
**Oligodendroglioma.
**Oligodendroglioma.
**[[Lymphoma]].
**[[Lymphoma]].
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Image:Oligodendroglioma1_high_mag.jpg | Oligodendroglioma high mag. (WC)
Image:Oligodendroglioma1_high_mag.jpg | Oligodendroglioma high mag. (WC)
Image:Oligodendroglioma1_low_mag.jpg | Oligodendroglioma low mag. (WC)
Image:Oligodendroglioma1_low_mag.jpg | Oligodendroglioma low mag. (WC)
File:Oligodendroglioma_discrete_invasion_HE.jpg | Discrete invasion in a oligodendroglioma. (WC/jensflorian)
File:Anaplastic_oligodendroglioma_minigemistocytes.jpg | Minigemistocytes and mitoses. (WC/jensflorian)
MAP2_anaplastic_oligodendroglioma.jpg | Perinuclear [[MAP2]] immunoreactivity in oligodendroglioma. (WC/jensflorian)
File:IDH1_R132H_in_anaplastic_ologodendroglioma.jpg | Demonstration of [[IDH-1|IDH1 R132H]] mutation in oligodendroglioma. (WC/jensflorian)
</gallery>
</gallery>
www:
www:
*[http://path.upmc.edu/cases/case713.html Oligodendroglioma - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case713.html Oligodendroglioma - several images (upmc.edu)].
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===Histologic grading===
===Histologic grading===
Come in two flavours:
Come in two flavours:
# WHO grade II.
# WHO CNS grade 2
#*This is most oligodendrogliomas.
#*This is most oligodendrogliomas.
# WHO grade III.
#*Without genetic testing for [[IDH-1]]/2 and LOH 1p/19q, the tumor is called Oligodendroglioma, NOS.
# WHO CNS grade 3
#*Features for calling high grade:<ref name=Ref_PSNP98>{{Ref PSNP|98}}</ref>
#*Features for calling high grade:<ref name=Ref_PSNP98>{{Ref PSNP|98}}</ref>
#**Endothelial hypertrophy.
#**Endothelial hypertrophy.
#***Plump/large endothelial cells.
#***Plump/large endothelial cells.
#**Necrosis.
#**Necrosis.
#**High mitotic rate (6 mitoses/10 HPF for whatever "HPF" means, see [[HPFitis]]).
#**High mitotic rate (6 or more mitoses/10 HPF of 0.55mm²).
#*Without genetic testing for [[IDH-1]]/2 and LOH 1p/19q, the tumor is called Anaplastic oligodendroglioma, NOS.


==IHC==
==IHC==
Features:
Features:
*MAP-2 +ve.<reF name=pmid12025943>{{cite journal |author=Suzuki SO, Kitai R, Llena J, Lee SC, Goldman JE, Shafit-Zagardo B |title=MAP-2e, a novel MAP-2 isoform, is expressed in gliomas and delineates tumor architecture and patterns of infiltration |journal=J. Neuropathol. Exp. Neurol. |volume=61 |issue=5 |pages=403–12 |year=2002 |month=May |pmid=12025943 |doi= |url=}}</ref>
*[[MAP2]] +ve.<reF name=pmid12025943>{{cite journal |author=Suzuki SO, Kitai R, Llena J, Lee SC, Goldman JE, Shafit-Zagardo B |title=MAP-2e, a novel MAP-2 isoform, is expressed in gliomas and delineates tumor architecture and patterns of infiltration |journal=J. Neuropathol. Exp. Neurol. |volume=61 |issue=5 |pages=403–12 |year=2002 |month=May |pmid=12025943 |doi= |url=}}</ref>
*GFAP +ve (variable).
*GFAP +ve (variable).
**Some subtypes +ve - should not be used to distinguish.<ref name=Ref_PSNP>{{Ref PSNP|98}}</ref>
**Some subtypes +ve - should not be used to distinguish.<ref name=Ref_PSNP>{{Ref PSNP|98}}</ref>
*EMA +ve.
*EMA +ve.
*IDH-1 +ve. (80%).
*IDH-1 (R132H) +ve (approx 85%).
*p53 -ve.
**18% +ve in one series of children,<ref name=pmid24805856>{{Cite journal  | last1 = Rodriguez | first1 = FJ. | last2 = Tihan | first2 = T. | last3 = Lin | first3 = D. | last4 = McDonald | first4 = W. | last5 = Nigro | first5 = J. | last6 = Feuerstein | first6 = B. | last7 = Jackson | first7 = S. | last8 = Cohen | first8 = K. | last9 = Burger | first9 = PC. | title = Clinicopathologic features of pediatric oligodendrogliomas: a series of 50 patients. | journal = Am J Surg Pathol | volume = 38 | issue = 8 | pages = 1058-70 | month = Aug | year = 2014 | doi = 10.1097/PAS.0000000000000221 | PMID = 24805856 }}</ref> 23% +ve in another series.<ref name=pmid23361281>{{Cite journal  | last1 = Sipayya | first1 = V. | last2 = Sharma | first2 = I. | last3 = Sharma | first3 = KC. | last4 = Singh | first4 = A. | title = Immunohistochemical expression of IDH1 in gliomas: a tissue microarray-based approach. | journal = J Cancer Res Ther | volume = 8 | issue = 4 | pages = 598-601 | month =  | year =  | doi = 10.4103/0973-1482.106567 | PMID = 23361281 }}</ref>
**Useful for differentiating ''astrocytoma'' vs. ''oligodendroglioma''.
*ATRX +ve (nuclear retention).
*Ki-67.
**Useful for differentiating ''astrocytoma'' vs. ''oligodendroglioma''.<ref>{{Cite journal  | last1 = Reuss | first1 = DE. | last2 = Sahm | first2 = F. | last3 = Schrimpf | first3 = D. | last4 = Wiestler | first4 = B. | last5 = Capper | first5 = D. | last6 = Koelsche | first6 = C. | last7 = Schweizer | first7 = L. | last8 = Korshunov | first8 = A. | last9 = Jones | first9 = DT. | title = ATRX and IDH1-R132H immunohistochemistry with subsequent copy number analysis and IDH sequencing as a basis for an "integrated" diagnostic approach for adult astrocytoma, oligodendroglioma and glioblastoma. | journal = Acta Neuropathol | volume = 129 | issue = 1 | pages = 133-46 | month = Jan | year = 2015 | doi = 10.1007/s00401-014-1370-3 | PMID = 25427834 }}</ref>
*H3K27me3 -ve (nuclear loss).<ref>{{Cite journal  | last1 = Filipski | first1 = K. | last2 = Braun | first2 = Y. | last3 = Zinke | first3 = J. | last4 = Roller | first4 = B. | last5 = Baumgarten | first5 = P. | last6 = Wagner | first6 = M. | last7 = Senft | first7 = C. | last8 = Zeiner | first8 = PS. | last9 = Ronellenfitsch | first9 = MW. | title = Lack of H3K27 trimethylation is associated with 1p/19q codeletion in diffuse gliomas. | journal = Acta Neuropathol | volume =  | issue =  | pages =  | month = May | year = 2019 | doi = 10.1007/s00401-019-02025-9 | PMID = 31065834 }}</ref>
*SOX10 +ve (up to 80%).<ref>{{Cite journal  | last1 = Bannykh | first1 = SI. | last2 = Stolt | first2 = CC. | last3 = Kim | first3 = J. | last4 = Perry | first4 = A. | last5 = Wegner | first5 = M. | title = Oligodendroglial-specific transcriptional factor SOX10 is ubiquitously expressed in human gliomas. | journal = J Neurooncol | volume = 76 | issue = 2 | pages = 115-27 | month = Jan | year = 2006 | doi = 10.1007/s11060-005-5533-x | PMID = 16205963 }}</ref>
*Olig2 +ve.
*H3 K27me3 nuclear loss in most cases.
*NOGO-A often +ve.
*p53 -ve or low expressed.
*Ki-67 (usu. >5% in CNS grade 2).
*May have neuronal "islands" (Synapto +ve, NeuN +ve).


==Molecular pathology==
==Molecular pathology==
Losses of 1p and 19q both helps with diagnosis and is prognostic:<ref name=pmid18565359>{{cite journal |author=Fontaine D, Vandenbos F, Lebrun C, Paquis V, Frenay M |title=[Diagnostic and prognostic values of 1p and 19q deletions in adult gliomas: critical review of the literature and implications in daily clinical practice] |language=French |journal=Rev. Neurol. (Paris) |volume=164 |issue=6-7 |pages=595–604 |year=2008 |pmid=18565359 |doi=10.1016/j.neurol.2008.04.002 |url=}}</ref>
Combined losses of 1p and 19q both and presence of IDH1 mutation in codon 132 or IDH2 mutation in codon 172 is required for final diagnosis and is prognostic and therapeutic relevant:<ref name=pmid18565359>{{cite journal |author=Fontaine D, Vandenbos F, Lebrun C, Paquis V, Frenay M |title=[Diagnostic and prognostic values of 1p and 19q deletions in adult gliomas: critical review of the literature and implications in daily clinical practice] |language=French |journal=Rev. Neurol. (Paris) |volume=164 |issue=6-7 |pages=595–604 |year=2008 |pmid=18565359 |doi=10.1016/j.neurol.2008.04.002 |url=}}</ref><ref>{{Cite journal  | last1 = Wiestler | first1 = B. | last2 = Capper | first2 = D. | last3 = Hovestadt | first3 = V. | last4 = Sill | first4 = M. | last5 = Jones | first5 = DT. | last6 = Hartmann | first6 = C. | last7 = Felsberg | first7 = J. | last8 = Platten | first8 = M. | last9 = Feiden | first9 = W. | title = Assessing CpG island methylator phenotype, 1p/19q codeletion, and MGMT promoter methylation from epigenome-wide data in the biomarker cohort of the NOA-04 trial. | journal = Neuro Oncol | volume = 16 | issue = 12 | pages = 1630-8 | month = Dec | year = 2014 | doi = 10.1093/neuonc/nou138 | PMID = 25028501 }}</ref><ref>{{Cite journal  | last1 = Cairncross | first1 = G. | last2 = Wang | first2 = M. | last3 = Shaw | first3 = E. | last4 = Jenkins | first4 = R. | last5 = Brachman | first5 = D. | last6 = Buckner | first6 = J. | last7 = Fink | first7 = K. | last8 = Souhami | first8 = L. | last9 = Laperriere | first9 = N. | title = Phase III trial of chemoradiotherapy for anaplastic oligodendroglioma: long-term results of RTOG 9402. | journal = J Clin Oncol | volume = 31 | issue = 3 | pages = 337-43 | month = Jan | year = 2013 | doi = 10.1200/JCO.2012.43.2674 | PMID = 23071247 }}</ref>
*Greater chemosensitivity  
*Greater chemosensitivity to PCV regimen.<ref>{{Cite journal  | last1 = Cairncross | first1 = G. | last2 = Wang | first2 = M. | last3 = Shaw | first3 = E. | last4 = Jenkins | first4 = R. | last5 = Brachman | first5 = D. | last6 = Buckner | first6 = J. | last7 = Fink | first7 = K. | last8 = Souhami | first8 = L. | last9 = Laperriere | first9 = N. | title = Phase III trial of chemoradiotherapy for anaplastic oligodendroglioma: long-term results of RTOG 9402. | journal = J Clin Oncol | volume = 31 | issue = 3 | pages = 337-43 | month = Jan | year = 2013 | doi = 10.1200/JCO.2012.43.2674 | PMID = 23071247 }}</ref>
*Better prognosis.
*Better prognosis compared to astrocytic tumors.<ref>{{Cite journal  | last1 = Mur | first1 = P. | last2 = Mollejo | first2 = M. | last3 = Ruano | first3 = Y. | last4 = de Lope | first4 = ÁR. | last5 = Fiaño | first5 = C. | last6 = García | first6 = JF. | last7 = Castresana | first7 = JS. | last8 = Hernández-Laín | first8 = A. | last9 = Rey | first9 = JA. | title = Codeletion of 1p and 19q determines distinct gene methylation and expression profiles in IDH-mutated oligodendroglial tumors. | journal = Acta Neuropathol | volume = 126 | issue = 2 | pages = 277-89 | month = Aug | year = 2013 | doi = 10.1007/s00401-013-1130-9 | PMID = 23689617 }}</ref>
*TERT promotor mutation present.
*CDKN2A homozygoud deletion in <10% of grade 3 tumours.
 
Note: If molecular testing fails, cases should be classified as Oligodendroglioma, NOS.
 
DDx:
*[[Neurocytoma]] also have perinuclear clearing and well-defined cellular borders.
**Pineocytomatous/neurocytic rosettes = (irregular) rosette with a large meshwork of fibers (neuropil) at the centre.
*Clear cell [[ependymoma]].
*[[Dysembryoplastic neuroepithelial tumour]].
*Oligodendroglial-like cells in [[Pilocytic astrocytoma]].
*Clear cell [[meningioma]]. (EMA +ve)
*Clear cell renal carcinoma. (Pan-CK +ve)
*Demyelinisation.
* [[Astrocytoma]]
** [[IDH-1]] mutant, but non-codeleted tumors with oligodendrogliom histologye are no longer classified as oligodendrogliomas on molecular basis. These tumors are classified as IDH-mutant astrocytoma.
* Diffuse leptomeningeal glioneuronal tumour (1p/19q codeletion present, but IDH wildtype)
 
Note:
*Consider Glioblastoma with oligo features when tumor is IDH1/2 wildtype and has no LOH 1p/19q and no ATRX loss.
*Dual-genotype astrocytoma/oligodendroglioma are very rare.


==See also==
==See also==
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[[Category:Neuropathology tumours]]
[[Category:Neuropathology tumours]]
[[Category:Diagnosis]]
[[Category:Diagnosis]]
[[Category:WHO grade II tumours]]
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