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Difference between revisions of "Oligodendroglioma"
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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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The fifth edition of WHO classification recognizes two tumor subtypes: | 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 2 (ICD-O: 9450/3). | ||
*Oligodendroglioma, IDH-mutant and 1p/19q-codeleted, WHO CNS Grade | *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> | 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> | ||
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*Some tumors may show a [[Polar Spongioblastoma|Spongioblastoma]]-like growth pattern. | *Some tumors may show a [[Polar Spongioblastoma|Spongioblastoma]]-like growth pattern. | ||
Anaplastic (grade | 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. | *"Significant" or "brisk" mitotic activity. | ||
**That means for most neuropathologists >= 6 mitoses per 10 HPF. | **That means for most neuropathologists >= 6 mitoses per 10 HPF. | ||
*Microvacular proliferation. | *Microvacular proliferation. | ||
*Necrosis. | *Necrosis. | ||
*Rarely multnucleated cells. | |||
Note: | Note: | ||
* 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> | * 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> | ||
**Also called minigemistocytes. | **Also called minigemistocytes. | ||
**The are usually strong GFAP+ve. | **The are usually strong GFAP+ve. | ||
Notes: | Notes: | ||
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#***Plump/large endothelial cells. | #***Plump/large endothelial cells. | ||
#**Necrosis. | #**Necrosis. | ||
#**High mitotic rate (6 mitoses/10 HPF | #**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. | #*Without genetic testing for [[IDH-1]]/2 and LOH 1p/19q, the tumor is called Anaplastic oligodendroglioma, NOS. | ||
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*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> | *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> | *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. | *p53 -ve or low expressed. | ||
*Ki-67 (usu. >5% in grade | *Ki-67 (usu. >5% in CNS grade 2). | ||
*May have neuronal "islands" (Synapto +ve, NeuN +ve). | *May have neuronal "islands" (Synapto +ve, NeuN +ve). | ||
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*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> | *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 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> | *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: | Note: | ||
*Consider | *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== | ||