@@ Line 1: / Line 1: @@
 {{DISPLAYTITLE:Oligodendroglioma, IDH-mutant and 1p/19q-codeleted}}
-[[CNS5:Table_of_Contents|Central Nervous System Tumours(WHO Classification, 5th ed.)]]
+[[CNS5:Table_of_Contents|Central Nervous System Tumours (WHO Classification, 5th ed.)]]
 {{Under Construction}}
@@ Line 11: / Line 11: @@
 Shashirekha Shetty, PhD, Director, Cytogenetics Laboratory, Center for Human Genetics Laboratory, University Hospitals
 ==WHO Classification of Disease==
@@ Line 35: / Line 34: @@
 |}
-==Definition / Description of Disease==
+==Related Terminology==
-A molecularly defined diffusely infiltrating glioma with IDH1 or IDH2 mutation and codeletion of chromosome arms 1p and 19q<ref name=":0">WHO Classification of Tumours Editorial Board. Central nervous system tumours. Lyon (France): International Agency for Research on Cancer; 2021. (WHO classification of tumours series, 5th ed.; vol. 6). <nowiki>https://publications.iarc.fr/601</nowiki>.</ref> .
-Oligodendrogliomas are graded morphologically as either CNS WHO grade 2 or CNS WHO grade 3.
-In rare cases where molecular studies are unable to be completed or have failed, tumors can be histologically diagnosed as Oligodendroglioma, NOS (not otherwise specified).
-==Synonyms / Terminology==
-Anaplastic oligodendroglioma (historical; now known as oligodendroglioma, IDH-mutant and 1p/19q-codeleted, CNS WHO grade 3).
-Oligoastrocytoma (discouraged; oligodendroglioma and astrocytoma are molecularly distinct entities. The diagnosis is reserved for rare cases where a dual genotype is identified, or where molecular testing could not be completed).
-==Epidemiology / Prevalence==
--         Epidemiological statistics should be interpreted with caution as oligodendroglioma is now molecularly defined
-*  A subset of tumor historically diagnosed as oligodendroglioma on morphological grounds may therefore not meet current definition
--         Oligodendrogliomas occur primarily in adults (median age 43 years for CNS WHO grade 2 and 50 years for CNS WHO grade 3)<ref name=":1">{{Cite journal|last=Ostrom|first=Quinn T.|last2=Cioffi|first2=Gino|last3=Gittleman|first3=Haley|last4=Patil|first4=Nirav|last5=Waite|first5=Kristin|last6=Kruchko|first6=Carol|last7=Barnholtz-Sloan|first7=Jill S.|date=2019-11-01|title=CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2012-2016|url=https://pubmed.ncbi.nlm.nih.gov/31675094|journal=Neuro-Oncology|volume=21|issue=Suppl 5|pages=v1–v100|doi=10.1093/neuonc/noz150|issn=1523-5866|pmc=6823730|pmid=31675094}}</ref>
-*  Slight male preponderance (M:F = 1.2:1<ref name=":1" />)
--         Low incidence worldwide
-*  Incidence is changing over time due to refined molecular definition
-**  Incidence rate (cases per 100,000 person-years) for histologically defined oligodendroglioma – 0.10% (Republic of Korea; <ref name=":2">{{Cite journal|last=Lee|first=Chang-Hyun|last2=Jung|first2=Kyu-Won|last3=Yoo|first3=Heon|last4=Park|first4=Sohee|last5=Lee|first5=Seung Hoon|date=2010-08|title=Epidemiology of primary brain and central nervous system tumors in Korea|url=https://pubmed.ncbi.nlm.nih.gov/20856664|journal=Journal of Korean Neurosurgical Society|volume=48|issue=2|pages=145–152|doi=10.3340/jkns.2010.48.2.145|issn=1598-7876|pmc=2941858|pmid=20856664}}</ref>), 0.50 (France <ref name=":3">{{Cite journal|last=Darlix|first=Amélie|last2=Zouaoui|first2=Sonia|last3=Rigau|first3=Valérie|last4=Bessaoud|first4=Faiza|last5=Figarella-Branger|first5=Dominique|last6=Mathieu-Daudé|first6=Hélène|last7=Trétarre|first7=Brigitte|last8=Bauchet|first8=Fabienne|last9=Duffau|first9=Hugues|date=2017-02|title=Epidemiology for primary brain tumors: a nationwide population-based study|url=https://pubmed.ncbi.nlm.nih.gov/27853959|journal=Journal of Neuro-Oncology|volume=131|issue=3|pages=525–546|doi=10.1007/s11060-016-2318-3|issn=1573-7373|pmid=27853959}}</ref>), 0.23 (USA 31675094<ref name=":1" />)
-**Incidence rate for histologically defined CNS WHO Grade 3 oligodendroglioma – 0.06% (Republic of Korea<ref name=":2" />), 0.39 (France <ref name=":3" />), 0.11 (USA<ref name=":1" />)
-*  CNS WHO grade 2 oligodendrogliomas account for 0.9% of primary brain tumors in US (PMID: 34608945)<ref name=":1" />
-*  CNS WHO grade 3 oligodendrogliomas account of primary brain tumors in the US(PMID: 34608945)<ref name=":1" />
-==Clinical Features==
--         Oligodendrogliomas are most often low-grade, slow growing tumors
-*  Tumors are frequently asymptomatic and are increasingly found incidentally on imaging for other indications<ref>{{Cite journal|last=Wijnenga|first=Maarten M. J.|last2=French|first2=Pim J.|last3=Dubbink|first3=Hendrikus J.|last4=Dinjens|first4=Winand N. M.|last5=Atmodimedjo|first5=Peggy N.|last6=Kros|first6=Johan M.|last7=Smits|first7=Marion|last8=Gahrmann|first8=Renske|last9=Rutten|first9=Geert-Jan|date=2018-01-10|title=The impact of surgery in molecularly defined low-grade glioma: an integrated clinical, radiological, and molecular analysis|url=https://pubmed.ncbi.nlm.nih.gov/29016833|journal=Neuro-Oncology|volume=20|issue=1|pages=103–112|doi=10.1093/neuonc/nox176|issn=1523-5866|pmc=5761503|pmid=29016833}}</ref>
--         Most commonly present with seizures<ref name=":4">{{Cite journal|last=Zetterling|first=Maria|last2=Berhane|first2=Luwam|last3=Alafuzoff|first3=Irina|last4=Jakola|first4=Asgeir S.|last5=Smits|first5=Anja|date=2017|title=Prognostic markers for survival in patients with oligodendroglial tumors; a single-institution review of 214 cases|url=https://pubmed.ncbi.nlm.nih.gov/29186201|journal=PloS One|volume=12|issue=11|pages=e0188419|doi=10.1371/journal.pone.0188419|issn=1932-6203|pmc=5706698|pmid=29186201}}</ref>
--         Can present with focal neurologic deficits or cognitive changes secondary to increased cranial pressure, especially in the high grade setting<ref name=":4" />
 {| class="wikitable"
-|'''Signs and Symptoms'''
+|+
-|Seizures<ref name=":4" />
+|Acceptable
+|N/A
-Headache
-Signs  of increased intracranial pressure
--           Focal neurologic deficits
--           Cognitive changes
-Asymptomatic
--           Increasingly an incidental finding  on neuroimaging (PMID: 29186201)
 |-
-|'''Laboratory Findings'''
+|Not Recommended
-|Not  applicable
+|Anaplastic oligodendroglioma, IDH-mutant and 1p/19q-codeleted
-|}
-==Sites of Involvement==
--         Approximately 60% of oligodendrogliomas occur within the frontal lobes with<ref name=":0" /><ref name=":1" />
-*  14-16% in the temporal lobe
-*  10-15% in the parietal lobe
-*  1-6% in the occipital lobe
-*  Less commonly basal ganglia / cerebellum brainstem
--         Leptomeningeal spread and gliomatosis cerebri pattern can rarely occur<ref>{{Cite journal|last=Andersen|first=Brian M.|last2=Miranda|first2=Caroline|last3=Hatzoglou|first3=Vaios|last4=DeAngelis|first4=Lisa M.|last5=Miller|first5=Alexandra M.|date=2019-05-21|title=Leptomeningeal metastases in glioma: The Memorial Sloan Kettering Cancer Center experience|url=https://pubmed.ncbi.nlm.nih.gov/31019097|journal=Neurology|volume=92|issue=21|pages=e2483–e2491|doi=10.1212/WNL.0000000000007529|issn=1526-632X|pmc=6541431|pmid=31019097}}</ref> <ref>{{Cite journal|last=Herrlinger|first=Ulrich|last2=Jones|first2=David T. W.|last3=Glas|first3=Martin|last4=Hattingen|first4=Elke|last5=Gramatzki|first5=Dorothee|last6=Stuplich|first6=Moritz|last7=Felsberg|first7=Jörg|last8=Bähr|first8=Oliver|last9=Gielen|first9=Gerrit H.|date=2016-02|title=Gliomatosis cerebri: no evidence for a separate brain tumor entity|url=https://pubmed.ncbi.nlm.nih.gov/26493382|journal=Acta Neuropathologica|volume=131|issue=2|pages=309–319|doi=10.1007/s00401-015-1495-z|issn=1432-0533|pmid=26493382}}</ref>
--         Rare spinal lesions have been reported but lack genotyping to confirm true oligodendroglioma<ref>{{Cite journal|last=Fountas|first=Kostas N.|last2=Karampelas|first2=Ioannis|last3=Nikolakakos|first3=Leonidas G.|last4=Troup|first4=E. Christopher|last5=Robinson|first5=Joe Sam|date=2005-02|title=Primary spinal cord oligodendroglioma: case report and review of the literature|url=https://pubmed.ncbi.nlm.nih.gov/15138790|journal=Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery|volume=21|issue=2|pages=171–175|doi=10.1007/s00381-004-0973-8|issn=0256-7040|pmid=15138790}}</ref> <ref>{{Cite journal|last=Hasturk|first=Askin Esen|last2=Gokce|first2=Emre Cemal|last3=Elbir|first3=Cagri|last4=Gel|first4=Gulce|last5=Canbay|first5=Suat|date=2017|title=A very rare spinal cord tumor primary spinal oligodendroglioma: A review of sixty cases in the literature|url=https://pubmed.ncbi.nlm.nih.gov/29021677|journal=Journal of Craniovertebral Junction & Spine|volume=8|issue=3|pages=253–262|doi=10.4103/jcvjs.JCVJS_1_17|issn=0974-8237|pmc=5634112|pmid=29021677}}</ref>
--         Extracranial metastasis exceedingly rare (CNS WHO grade 3)<ref>{{Cite journal|last=Merrell|first=Ryan|last2=Nabors|first2=L. Burton|last3=Perry|first3=Arie|last4=Palmer|first4=Cheryl Ann|date=2006-11|title=1p/19q chromosome deletions in metastatic oligodendroglioma|url=https://pubmed.ncbi.nlm.nih.gov/16710746|journal=Journal of Neuro-Oncology|volume=80|issue=2|pages=203–207|doi=10.1007/s11060-006-9179-0|issn=0167-594X|pmid=16710746}}</ref> <ref>{{Cite journal|last=Singh|first=Vikas K.|last2=Singh|first2=Shipra|last3=Bhupalam|first3=Leela|date=2019-07|title=Anaplastic oligodendroglioma metastasizing to the bone marrow: a unique case report and literature review|url=https://pubmed.ncbi.nlm.nih.gov/30526175|journal=The International Journal of Neuroscience|volume=129|issue=7|pages=722–728|doi=10.1080/00207454.2018.1557165|issn=1563-5279|pmid=30526175}}</ref> <ref>{{Cite journal|last=Burgy|first=Mickaël|last2=Chenard|first2=Marie-Pierre|last3=Noël|first3=Georges|last4=Bourahla|first4=Khalil|last5=Schott|first5=Roland|date=2019-06-28|title=Bone metastases from a 1p/19q codeleted and IDH1-mutant anaplastic oligodendroglioma: a case report|url=https://pubmed.ncbi.nlm.nih.gov/31248444|journal=Journal of Medical Case Reports|volume=13|issue=1|pages=202|doi=10.1186/s13256-019-2061-4|issn=1752-1947|pmc=6598291|pmid=31248444}}</ref>
-==Morphologic Features==
--         Classically consist of cells with round, monomorphous nuclei with stippled chromatin and perinuclear halos (artifactual fried-egg appearance)
--         Intervening delicate “chicken wire” vasculature
--         Can contain GFAP-positive minigemistocytes
--         Often contain microcalcifications, especially in low-grade tumors<ref name=":0" />
-==Immunophenotype==
-<br />
-{| class="wikitable sortable"
-|-
-!Finding!!Marker
-|-
-|Positive (universal)||Retained nuclear ATRX<ref name=":5">{{Cite journal|last=Liu|first=Xiao-Yang|last2=Gerges|first2=Noha|last3=Korshunov|first3=Andrey|last4=Sabha|first4=Nesrin|last5=Khuong-Quang|first5=Dong-Anh|last6=Fontebasso|first6=Adam M.|last7=Fleming|first7=Adam|last8=Hadjadj|first8=Djihad|last9=Schwartzentruber|first9=Jeremy|date=2012-11|title=Frequent ATRX mutations and loss of expression in adult diffuse astrocytic tumors carrying IDH1/IDH2 and TP53 mutations|url=https://pubmed.ncbi.nlm.nih.gov/22886134|journal=Acta Neuropathologica|volume=124|issue=5|pages=615–625|doi=10.1007/s00401-012-1031-3|issn=1432-0533|pmid=22886134}}</ref>, OLIG2<ref>{{Cite journal|last=Ligon|first=Keith L.|last2=Alberta|first2=John A.|last3=Kho|first3=Alvin T.|last4=Weiss|first4=Jennifer|last5=Kwaan|first5=Mary R.|last6=Nutt|first6=Catherine L.|last7=Louis|first7=David N.|last8=Stiles|first8=Charles D.|last9=Rowitch|first9=David H.|date=2004-05|title=The oligodendroglial lineage marker OLIG2 is universally expressed in diffuse gliomas|url=https://pubmed.ncbi.nlm.nih.gov/15198128|journal=Journal of Neuropathology and Experimental Neurology|volume=63|issue=5|pages=499–509|doi=10.1093/jnen/63.5.499|issn=0022-3069|pmid=15198128}}</ref>, S100<ref>{{Cite journal|last=Reifenberger|first=G.|last2=Szymas|first2=J.|last3=Wechsler|first3=W.|date=1987|title=Differential expression of glial- and neuronal-associated antigens in human tumors of the central and peripheral nervous system|url=https://pubmed.ncbi.nlm.nih.gov/3314309|journal=Acta Neuropathologica|volume=74|issue=2|pages=105–123|doi=10.1007/BF00692841|issn=0001-6322|pmid=3314309}}</ref>, MAP2<ref>{{Cite journal|last=Blümcke|first=I.|last2=Becker|first2=A. J.|last3=Normann|first3=S.|last4=Hans|first4=V.|last5=Riederer|first5=B. M.|last6=Krajewski|first6=S.|last7=Wiestler|first7=O. D.|last8=Reifenberger|first8=G.|date=2001-10|title=Distinct expression pattern of microtubule-associated protein-2 in human oligodendrogliomas and glial precursor cells|url=https://pubmed.ncbi.nlm.nih.gov/11589429|journal=Journal of Neuropathology and Experimental Neurology|volume=60|issue=10|pages=984–993|doi=10.1093/jnen/60.10.984|issn=0022-3069|pmid=11589429}}</ref>, SOX10<ref>{{Cite journal|last=Bannykh|first=Sergei I.|last2=Stolt|first2=C. Claus|last3=Kim|first3=Jung|last4=Perry|first4=Arie|last5=Wegner|first5=Michael|date=2006-01|title=Oligodendroglial-specific transcriptional factor SOX10 is ubiquitously expressed in human gliomas|url=https://pubmed.ncbi.nlm.nih.gov/16205963|journal=Journal of Neuro-Oncology|volume=76|issue=2|pages=115–127|doi=10.1007/s11060-005-5533-x|issn=0167-594X|pmid=16205963}}</ref>
-|-
-|Positive (subset)||Most positive for IDH1 p.R132H mutation (smaller subset lacking  staining have non-canonical IDH mutation, <10%)<ref>{{Cite journal|last=Capper|first=David|last2=Zentgraf|first2=Hanswalter|last3=Balss|first3=Jörg|last4=Hartmann|first4=Christian|last5=von Deimling|first5=Andreas|date=2009-11|title=Monoclonal antibody specific for IDH1 R132H mutation|url=https://pubmed.ncbi.nlm.nih.gov/19798509|journal=Acta Neuropathologica|volume=118|issue=5|pages=599–601|doi=10.1007/s00401-009-0595-z|issn=1432-0533|pmid=19798509}}</ref>
-Synaptophysin (cytoplasmic dot-like pattern<ref>{{Cite journal|last=Perry|first=Arie|last2=Burton|first2=Stephanie S.|last3=Fuller|first3=Gregory N.|last4=Robinson|first4=Christopher A.|last5=Palmer|first5=Cheryl A.|last6=Resch|first6=Lothar|last7=Bigio|first7=Eileen H.|last8=Gujrati|first8=Meena|last9=Rosenblum|first9=Marc K.|date=2010-08|title=Oligodendroglial neoplasms with ganglioglioma-like maturation: a diagnostic pitfall|url=https://pubmed.ncbi.nlm.nih.gov/20464403|journal=Acta Neuropathologica|volume=120|issue=2|pages=237–252|doi=10.1007/s00401-010-0695-9|issn=1432-0533|pmc=2892612|pmid=20464403}}</ref>)
-|-
-|Negative (universal)||Lack diffuse p53<ref name=":5" />
-|-
-|Negative (subset)||N/A
 |}
@@ Line 208: / Line 108: @@
+'''Add content below into table above'''
 -         Oligodendrogliomas are defined by a t(1;19)(q10;p10) rearrangement that results in 1p/19q whole-arm codeletion
@@ Line 236: / Line 136: @@
 {| class="wikitable sortable"
 |-
-!Chr #!!'''Gain, Loss, Amp, LOH'''!!'''Minimal Region Cytoband and/or Genomic Coordinates [Genome Build; Size]'''!!'''Relevant Gene(s)'''
+!Chr #!!Gain, Loss, Amp, LOH!!Minimal Region Cytoband and/or Genomic Coordinates [Genome Build; Size]!!Relevant Gene(s)
-!'''Diagnostic, Prognostic, and Therapeutic Significance - D, P, T'''
+!Diagnostic, Prognostic, and Therapeutic Significance - D, P, T
-!'''Established Clinical Significance Per Guidelines - Yes or No (Source)'''
+!Established Clinical Significance Per Guidelines - Yes or No (Source)
-!'''Clinical Relevance Details/Other Notes'''
+!Clinical Relevance Details/Other Notes
 |-
 |<span class="blue-text">EXAMPLE:</span>
@@ Line 288: / Line 188: @@
+'''Add content below into table above'''
 Put your text here and fill in the table
@@ Line 317: / Line 217: @@
 !Chromosomal Pattern
 !Molecular Pathogenesis
-!'''Prevalence -'''
+!Prevalence -
-'''Common >20%, Recurrent 5-20% or Rare <5% (Disease)'''
+Common >20%, Recurrent 5-20% or Rare <5% (Disease)
-!'''Diagnostic, Prognostic, and Therapeutic Significance - D, P, T'''
+!Diagnostic, Prognostic, and Therapeutic Significance - D, P, T
-!'''Established Clinical Significance Per Guidelines - Yes or No (Source)'''
+!Established Clinical Significance Per Guidelines - Yes or No (Source)
-!'''Clinical Relevance Details/Other Notes'''
+!Clinical Relevance Details/Other Notes
 |-
 |<span class="blue-text">EXAMPLE:</span>
@@ Line 349: / Line 249: @@
+'''Add content below into table above'''
 Put your text here
@@ Line 372: / Line 272: @@
 {| class="wikitable sortable"
 |-
-!Gene!!'''Genetic Alteration'''!!'''Tumor Suppressor Gene, Oncogene, Other'''!!'''Prevalence -'''
+!Gene!!Genetic Alteration!!Tumor Suppressor Gene, Oncogene, Other!!Prevalence -
-'''Common >20%, Recurrent 5-20% or Rare <5% (Disease)'''
+Common >20%, Recurrent 5-20% or Rare <5% (Disease)
-!'''Diagnostic, Prognostic, and Therapeutic Significance - D, P, T  '''
+!Diagnostic, Prognostic, and Therapeutic Significance - D, P, T
-!'''Established Clinical Significance Per Guidelines - Yes or No (Source)'''
+!Established Clinical Significance Per Guidelines - Yes or No (Source)
-!'''Clinical Relevance Details/Other Notes'''
+!Clinical Relevance Details/Other Notes
 |-
 |<span class="blue-text">EXAMPLE:</span>''EGFR''
@@ Line 416: / Line 316: @@
+'''Add content below into table above'''
 Put your text here and fill in the table
 {| class="wikitable sortable"
 |-
-!Gene; Genetic Alteration!!'''Presumed Mechanism (Tumor Suppressor Gene [TSG] / Oncogene / Other)'''!!'''Prevalence (COSMIC /  TCGA / Other)'''!!'''Concomitant Mutations'''!!'''Mutually Exclusive Mutations'''
+!Gene; Genetic Alteration!!Presumed Mechanism (Tumor Suppressor Gene [TSG] / Oncogene / Other)!!Prevalence (COSMIC /  TCGA / Other)!!Concomitant Mutations!!Mutually Exclusive Mutations
-!'''Diagnostic Significance (Yes, No or Unknown)'''
+!Diagnostic Significance (Yes, No or Unknown)
 !Prognostic Significance (Yes, No or Unknown)
 !Therapeutic Significance (Yes, No or Unknown)
@@ Line 507: / Line 407: @@
 |}
 Note: A more extensive list of mutations can be found in cBioportal (https://www.cbioportal.org/), COSMIC (https://cancer.sanger.ac.uk/cosmic), ICGC (https://dcc.icgc.org/) and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content.
 ==Epigenomic Alterations==
@@ Line 559: / Line 458: @@
 This disease is <u>defined/characterized</u> as detailed below:
-* A molecularly defined diffusely infiltrating glioma with IDH1 or IDH2 mutation and codeletion of chromosome arms 1p and 19q<ref name=":0" /> .
+*Can be called anaplastic oligodendroglioma (historical; now known as oligodendroglioma, IDH-mutant and 1p/19q-codeleted, CNS WHO grade 3). It is discouraged to call this entity oligoastrocytoma (oligodendroglioma and astrocytoma are molecularly distinct entities. The diagnosis is reserved for rare cases where a dual genotype is identified, or where molecular testing could not be completed).
-* Oligodendrogliomas are graded morphologically as either CNS WHO grade 2 or CNS WHO grade 3.
+*A molecularly defined diffusely infiltrating glioma with IDH1 or IDH2 mutation and codeletion of chromosome arms 1p and 19q<ref name=":0">WHO Classification of Tumours Editorial Board. Central nervous system tumours. Lyon (France): International Agency for Research on Cancer; 2021. (WHO classification of tumours series, 5th ed.; vol. 6). <nowiki>https://publications.iarc.fr/601</nowiki>.</ref> .
-* In rare cases where molecular studies are unable to be completed or have failed, tumors can be histologically diagnosed as Oligodendroglioma, NOS (not otherwise specified).
+*Oligodendrogliomas are graded morphologically as either CNS WHO grade 2 or CNS WHO grade 3.
+*In rare cases where molecular studies are unable to be completed or have failed, tumors can be histologically diagnosed as Oligodendroglioma, NOS (not otherwise specified).
 The <u>epidemiology/prevalence</u> of this disease is detailed below:
-* Epidemiological statistics should be interpreted with caution as oligodendroglioma is now molecularly defined.
+*Epidemiological statistics should be interpreted with caution as oligodendroglioma is now molecularly defined.
-** A subset of tumor historically diagnosed as oligodendroglioma on morphological grounds may therefore not meet current definition
+**A subset of tumor historically diagnosed as oligodendroglioma on morphological grounds may therefore not meet current definition
-* Oligodendrogliomas occur primarily in adults (median age 43 years for CNS WHO grade 2 and 50 years for CNS WHO grade 3)<ref name=":1" />
+*Oligodendrogliomas occur primarily in adults (median age 43 years for CNS WHO grade 2 and 50 years for CNS WHO grade 3)<ref name=":1">{{Cite journal|last=Ostrom|first=Quinn T.|last2=Cioffi|first2=Gino|last3=Gittleman|first3=Haley|last4=Patil|first4=Nirav|last5=Waite|first5=Kristin|last6=Kruchko|first6=Carol|last7=Barnholtz-Sloan|first7=Jill S.|date=2019-11-01|title=CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2012-2016|url=https://pubmed.ncbi.nlm.nih.gov/31675094|journal=Neuro-Oncology|volume=21|issue=Suppl 5|pages=v1–v100|doi=10.1093/neuonc/noz150|issn=1523-5866|pmc=6823730|pmid=31675094}}</ref>
-** Slight male preponderance (M:F = 1.2:1<ref name=":1" />)
+**Slight male preponderance (M:F = 1.2:1<ref name=":1" />)
-* Low incidence worldwide
+*Low incidence worldwide
-** Incidence is changing over time due to refined molecular definition
+**Incidence is changing over time due to refined molecular definition
-*** Incidence rate (cases per 100,000 person-years) for histologically defined oligodendroglioma – 0.10% (Republic of Korea; <ref name=":2" />), 0.50 (France <ref name=":3" />), 0.23 (USA 31675094<ref name=":1" />)
+***Incidence rate (cases per 100,000 person-years) for histologically defined oligodendroglioma – 0.10% (Republic of Korea; <ref name=":2">{{Cite journal|last=Lee|first=Chang-Hyun|last2=Jung|first2=Kyu-Won|last3=Yoo|first3=Heon|last4=Park|first4=Sohee|last5=Lee|first5=Seung Hoon|date=2010-08|title=Epidemiology of primary brain and central nervous system tumors in Korea|url=https://pubmed.ncbi.nlm.nih.gov/20856664|journal=Journal of Korean Neurosurgical Society|volume=48|issue=2|pages=145–152|doi=10.3340/jkns.2010.48.2.145|issn=1598-7876|pmc=2941858|pmid=20856664}}</ref>), 0.50 (France <ref name=":3">{{Cite journal|last=Darlix|first=Amélie|last2=Zouaoui|first2=Sonia|last3=Rigau|first3=Valérie|last4=Bessaoud|first4=Faiza|last5=Figarella-Branger|first5=Dominique|last6=Mathieu-Daudé|first6=Hélène|last7=Trétarre|first7=Brigitte|last8=Bauchet|first8=Fabienne|last9=Duffau|first9=Hugues|date=2017-02|title=Epidemiology for primary brain tumors: a nationwide population-based study|url=https://pubmed.ncbi.nlm.nih.gov/27853959|journal=Journal of Neuro-Oncology|volume=131|issue=3|pages=525–546|doi=10.1007/s11060-016-2318-3|issn=1573-7373|pmid=27853959}}</ref>), 0.23 (USA 31675094<ref name=":1" />)
-*** Incidence rate for histologically defined CNS WHO Grade 3 oligodendroglioma – 0.06% (Republic of Korea<ref name=":2" />), 0.39 (France <ref name=":3" />), 0.11 (USA<ref name=":1" />)
+***Incidence rate for histologically defined CNS WHO Grade 3 oligodendroglioma – 0.06% (Republic of Korea<ref name=":2" />), 0.39 (France <ref name=":3" />), 0.11 (USA<ref name=":1" />)
-** CNS WHO grade 2 oligodendrogliomas account for 0.9% of primary brain tumors in US (PMID: 34608945)<ref name=":1" />
+**CNS WHO grade 2 oligodendrogliomas account for 0.9% of primary brain tumors in US (PMID: 34608945)<ref name=":1" />
-** CNS WHO grade 3 oligodendrogliomas account of primary brain tumors in the US(PMID: 34608945)<ref name=":1" />
+**CNS WHO grade 3 oligodendrogliomas account of primary brain tumors in the US(PMID: 34608945)<ref name=":1" />
 The <u>clinical features</u> of this disease are detailed below:
-Signs and symptoms -
+Oligodendrogliomas are most often low-grade, slow growing tumors
-Laboratory findings -
+*Tumors are frequently asymptomatic and are increasingly found incidentally on imaging for other indications<ref name=":20">{{Cite journal|last=Wijnenga|first=Maarten M. J.|last2=French|first2=Pim J.|last3=Dubbink|first3=Hendrikus J.|last4=Dinjens|first4=Winand N. M.|last5=Atmodimedjo|first5=Peggy N.|last6=Kros|first6=Johan M.|last7=Smits|first7=Marion|last8=Gahrmann|first8=Renske|last9=Rutten|first9=Geert-Jan|date=2018-01-10|title=The impact of surgery in molecularly defined low-grade glioma: an integrated clinical, radiological, and molecular analysis|url=https://pubmed.ncbi.nlm.nih.gov/29016833|journal=Neuro-Oncology|volume=20|issue=1|pages=103–112|doi=10.1093/neuonc/nox176|issn=1523-5866|pmc=5761503|pmid=29016833}}</ref>
+*Most commonly present with seizures<ref name=":4">{{Cite journal|last=Zetterling|first=Maria|last2=Berhane|first2=Luwam|last3=Alafuzoff|first3=Irina|last4=Jakola|first4=Asgeir S.|last5=Smits|first5=Anja|date=2017|title=Prognostic markers for survival in patients with oligodendroglial tumors; a single-institution review of 214 cases|url=https://pubmed.ncbi.nlm.nih.gov/29186201|journal=PloS One|volume=12|issue=11|pages=e0188419|doi=10.1371/journal.pone.0188419|issn=1932-6203|pmc=5706698|pmid=29186201}}</ref>
+*Can present with focal neurologic deficits or cognitive changes secondary to increased cranial pressure, especially in the high-grade setting<ref name=":4" />
+*Signs and symptoms - Seizures<ref name=":4" />; Headache; Signs of increased intracranial pressure (Focal neurologic deficits, Cognitive changes); Asymptomatic (increasingly an incidental finding on neuroimaging<ref name=":4" />)
+*Laboratory findings - Not applicable
 The <u>sites of involvement</u> of this disease are detailed below:
+*Approximately 60% of oligodendrogliomas occur within the frontal lobes with
+**14-16% in the temporal lobe
+**10-15% in the parietal lobe
+**1-6% in the occipital lobe
+**Less commonly basal ganglia / cerebellum brainstem
+*Leptomeningeal spread and gliomatosis cerebri pattern can rarely occur
+*Rare spinal lesions have been reported but lack genotyping to confirm true oligodendroglioma
+*Extracranial metastasis exceedingly rare (CNS WHO grade 3)
 The <u>morphologic features</u> of this disease are detailed below:
+*Classically consist of cells with round, monomorphous nuclei with stippled chromatin and perinuclear halos (artifactual fried-egg appearance)
+**Intervening delicate “chicken wire” vasculature
+**Can contain GFAP-positive minigemistocytes
+**Often contain microcalcifications, especially in low-grade tumors<ref name=":0" />
 The <u>immunophenotype</u> of this disease is detailed below:
-Positive (universal) -
+*Positive (universal) - Retained nuclear ATRX<ref name=":5">{{Cite journal|last=Liu|first=Xiao-Yang|last2=Gerges|first2=Noha|last3=Korshunov|first3=Andrey|last4=Sabha|first4=Nesrin|last5=Khuong-Quang|first5=Dong-Anh|last6=Fontebasso|first6=Adam M.|last7=Fleming|first7=Adam|last8=Hadjadj|first8=Djihad|last9=Schwartzentruber|first9=Jeremy|date=2012-11|title=Frequent ATRX mutations and loss of expression in adult diffuse astrocytic tumors carrying IDH1/IDH2 and TP53 mutations|url=https://pubmed.ncbi.nlm.nih.gov/22886134|journal=Acta Neuropathologica|volume=124|issue=5|pages=615–625|doi=10.1007/s00401-012-1031-3|issn=1432-0533|pmid=22886134}}</ref>, OLIG2<ref name=":21">{{Cite journal|last=Ligon|first=Keith L.|last2=Alberta|first2=John A.|last3=Kho|first3=Alvin T.|last4=Weiss|first4=Jennifer|last5=Kwaan|first5=Mary R.|last6=Nutt|first6=Catherine L.|last7=Louis|first7=David N.|last8=Stiles|first8=Charles D.|last9=Rowitch|first9=David H.|date=2004-05|title=The oligodendroglial lineage marker OLIG2 is universally expressed in diffuse gliomas|url=https://pubmed.ncbi.nlm.nih.gov/15198128|journal=Journal of Neuropathology and Experimental Neurology|volume=63|issue=5|pages=499–509|doi=10.1093/jnen/63.5.499|issn=0022-3069|pmid=15198128}}</ref>, S100<ref name=":22">{{Cite journal|last=Reifenberger|first=G.|last2=Szymas|first2=J.|last3=Wechsler|first3=W.|date=1987|title=Differential expression of glial- and neuronal-associated antigens in human tumors of the central and peripheral nervous system|url=https://pubmed.ncbi.nlm.nih.gov/3314309|journal=Acta Neuropathologica|volume=74|issue=2|pages=105–123|doi=10.1007/BF00692841|issn=0001-6322|pmid=3314309}}</ref>, MAP2<ref name=":23">{{Cite journal|last=Blümcke|first=I.|last2=Becker|first2=A. J.|last3=Normann|first3=S.|last4=Hans|first4=V.|last5=Riederer|first5=B. M.|last6=Krajewski|first6=S.|last7=Wiestler|first7=O. D.|last8=Reifenberger|first8=G.|date=2001-10|title=Distinct expression pattern of microtubule-associated protein-2 in human oligodendrogliomas and glial precursor cells|url=https://pubmed.ncbi.nlm.nih.gov/11589429|journal=Journal of Neuropathology and Experimental Neurology|volume=60|issue=10|pages=984–993|doi=10.1093/jnen/60.10.984|issn=0022-3069|pmid=11589429}}</ref>, SOX10<ref name=":24">{{Cite journal|last=Bannykh|first=Sergei I.|last2=Stolt|first2=C. Claus|last3=Kim|first3=Jung|last4=Perry|first4=Arie|last5=Wegner|first5=Michael|date=2006-01|title=Oligodendroglial-specific transcriptional factor SOX10 is ubiquitously expressed in human gliomas|url=https://pubmed.ncbi.nlm.nih.gov/16205963|journal=Journal of Neuro-Oncology|volume=76|issue=2|pages=115–127|doi=10.1007/s11060-005-5533-x|issn=0167-594X|pmid=16205963}}</ref>
+*Positive (subset) - Most positive for IDH1 p.R132H mutation (smaller subset lacking staining have non-canonical IDH mutation, <10%)<ref name=":25">{{Cite journal|last=Capper|first=David|last2=Zentgraf|first2=Hanswalter|last3=Balss|first3=Jörg|last4=Hartmann|first4=Christian|last5=von Deimling|first5=Andreas|date=2009-11|title=Monoclonal antibody specific for IDH1 R132H mutation|url=https://pubmed.ncbi.nlm.nih.gov/19798509|journal=Acta Neuropathologica|volume=118|issue=5|pages=599–601|doi=10.1007/s00401-009-0595-z|issn=1432-0533|pmid=19798509}}</ref>, Synaptophysin (cytoplasmic dot-like pattern<ref name=":26">{{Cite journal|last=Perry|first=Arie|last2=Burton|first2=Stephanie S.|last3=Fuller|first3=Gregory N.|last4=Robinson|first4=Christopher A.|last5=Palmer|first5=Cheryl A.|last6=Resch|first6=Lothar|last7=Bigio|first7=Eileen H.|last8=Gujrati|first8=Meena|last9=Rosenblum|first9=Marc K.|date=2010-08|title=Oligodendroglial neoplasms with ganglioglioma-like maturation: a diagnostic pitfall|url=https://pubmed.ncbi.nlm.nih.gov/20464403|journal=Acta Neuropathologica|volume=120|issue=2|pages=237–252|doi=10.1007/s00401-010-0695-9|issn=1432-0533|pmc=2892612|pmid=20464403}}</ref>)
-Positive (majority) -
+*Negative (universal) - Lack diffuse p53<ref name=":5" />
+*Negative (subset) - N/A
-Positive (subset) -
-Negative (universal) -
-Negative (subset) -
 ==Links==

CNS5:Oligodendroglioma, IDH-mutant and 1p/19q-codeleted: Difference between revisions