Difference between revisions of "Oligodendroglioma classification"

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! style="background:#4479BA; color: #FFFFFF;" align="center" + |Defining* or characteristic molecular genetic features
! style="background:#4479BA; color: #FFFFFF;" align="center" + |Defining* or characteristic molecular genetic features
| colspan="2" style="background:#DCDCDC;" align="center" + |'''Astrocytic tumors'''
| colspan="3" style="background:#DCDCDC;" align="center" + |'''Astrocytic tumors'''
|[[Diffuse astrocytoma]], [[IDH1|IDH]]-[[mutant]]
|[[Diffuse astrocytoma]], [[IDH1|IDH]]-[[mutant]]
Line 50: Line 49:
|[[H3F3A|H3]] K27M [[mutation]]*
|[[H3F3A|H3]] K27M [[mutation]]*
| colspan="2" style="background:#DCDCDC;" align="center" + |'''Oligodendroglial tumors'''
| colspan="3" style="background:#DCDCDC;" align="center" + |'''Oligodendroglial tumors'''
|[[Oligodendroglioma]], [[IDH1|IDH]]-[[mutant]] and [[1p36 deletion syndrome|1p]]/19q-codeleted
|[[Oligodendroglioma]], [[IDH1|IDH]]-[[mutant]] and [[1p36 deletion syndrome|1p]]/19q-codeleted

Latest revision as of 00:43, 15 June 2019

Oligodendroglioma Microchapters


Patient Information


Historical Perspective




Differentiating Oligodendroglioma from other Diseases

Epidemiology & Demographics

Risk Factors


Natural History, Complications, and Prognosis



History & Symptoms

Physical Examination

Laboratory Findings

Chest X Ray




Other Imaging Findings

Other Diagnostic Studies


Medical Therapy


Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

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Oligodendroglioma classification in the news

Blogs on Oligodendroglioma classification

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Risk calculators and risk factors for Oligodendroglioma classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sara Mohsin, M.B.B.S.[2]Sujit Routray, M.D. [3]


According to the old 2007 WHO classification of the central nervous system tumors, oligodendrogliomas were divided into five subtypes: oligodendroglioma (OII), anaplastic oligodendroglioma (OIII), oligoastrocytoma (OAII), anaplastic oligoastrocytoma (OAIII), and glioblastoma with oligodendroglioma component (GBMo). But the new 2016 edition of WHO classification of gliomas is based not only on histopathologic appearance but also on well-established molecular parameters, and oligodendroglial tumors are now more narrowly defined by molecular diagnostics to include only those diffuse gliomas having both a mutation in isocitrate dehydrogenase type 1 (IDH1) or type 2 (IDH2) and codeletion of chromosomes 1p and 19q. This new pattern of classification divides oligodendrogliomas into grade II tumors including oligodendroglioma IDH-mutant and 1p/19q-codeleted, oligodendroglioma NOS, oligoastrocytoma NOS, and grade III tumors including anaplasticoligodendroglioma IDH-mutant and 1p/19q-codeleted, anaplastic oligodendroglioma NOS, and anaplastic oligoastrocytoma NOS.


2016 World Health Organization (WHO) classification of diffuse astrocytic and oligodendroglial tumors
Tumor classification Tumor grade Defining* or characteristic molecular genetic features
Astrocytic tumors
Diffuse astrocytoma, IDH-mutant II IDH1/2 mutation*, TP53 mutation, ATRX mutation
Diffuse astrocytoma, IDH-wildtype II No IDH1/2 mutation
Anaplastic astrocytoma, IDH-mutant III IDH1/2 mutation*, TP53 mutation, ATRX mutation
Anaplastic astrocytoma, IDH-wildtype III No IDH1/2 mutation
Glioblastoma, IDH-mutant IV IDH1/2 mutation*, TP53 mutation, ATRX mutation
Glioblastoma, IDH-wildtype IV No IDH1/2 mutation, TERT promoter mutations
Glioblastoma, NOS IV Genetic testing not done or inconclusive
Midline diffuse glioma, H3 K27M-mutant IV H3 K27M mutation*
Oligodendroglial tumors
Oligodendroglioma, IDH-mutant and 1p/19q-codeleted II IDH1/2 mutation*, 1p/19q-codeletion*, no ATRX mutation, TERT promoter mutations
Oligodendroglioma, NOS II Genetic testing not done or inconclusive
Oligoastrocytoma, NOS II Genetic testing not done or inconclusive
Anaplastic oligodendroglioma, IDH-mutant and 1p/19q-codeleted III IDH1/2 mutation*, 1p/19q-codeletion*, no ATRX mutation, TERT promoter mutations
Anaplastic oligodendroglioma, NOS III Genetic testing not done or inconclusive
Anaplastic oligoastrocytoma, NOS III Genetic testing not done or inconclusive

IDH: isocitrate dehydrogenase; NOS: not otherwise specified

Data from: WHO classification of tumors of the central nervous system, revised 4th ed, Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (Eds), IARC, Lyon 2016

WHO grade II
WHO grade III
WHO grade IV


  1. Perry A (2016). "WHO's arrived in 2016! An updated weather forecast for integrated brain tumor diagnosis". Brain Tumor Pathol. 33 (3): 157–60. doi:10.1007/s10014-016-0266-4. PMID 27295314.
  2. Louis DN, Perry A, Reifenberger G, von Deimling A, Figarella-Branger D, Cavenee WK; et al. (2016). "The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary". Acta Neuropathol. 131 (6): 803–20. doi:10.1007/s00401-016-1545-1. PMID 27157931.
  3. Perry A, Burton SS, Fuller GN, Robinson CA, Palmer CA, Resch L; et al. (2010). "Oligodendroglial neoplasms with ganglioglioma-like maturation: a diagnostic pitfall". Acta Neuropathol. 120 (2): 237–52. doi:10.1007/s00401-010-0695-9. PMC 2892612. PMID 20464403.
  4. Teo JG, Gultekin SH, Bilsky M, Gutin P, Rosenblum MK (1999). "A distinctive glioneuronal tumor of the adult cerebrum with neuropil-like (including "rosetted") islands: report of 4 cases". Am J Surg Pathol. 23 (5): 502–10. PMID 10328080.
  5. Rodriguez FJ, Scheithauer BW, Robbins PD, Burger PC, Hessler RB, Perry A; et al. (2007). "Ependymomas with neuronal differentiation: a morphologic and immunohistochemical spectrum". Acta Neuropathol. 113 (3): 313–24. doi:10.1007/s00401-006-0153-x. PMID 17061076.
  6. Brat DJ, Hirose Y, Cohen KJ, Feuerstein BG, Burger PC (2000). "Astroblastoma: clinicopathologic features and chromosomal abnormalities defined by comparative genomic hybridization". Brain Pathol. 10 (3): 342–52. PMID 10885653.
  7. Jouvet A, Fauchon F, Liberski P, Saint-Pierre G, Didier-Bazes M, Heitzmann A; et al. (2003). "Papillary tumor of the pineal region". Am J Surg Pathol. 27 (4): 505–12. PMID 12657936.
  8. Wang M, Tihan T, Rojiani AM, Bodhireddy SR, Prayson RA, Iacuone JJ; et al. (2005). "Monomorphous angiocentric glioma: a distinctive epileptogenic neoplasm with features of infiltrating astrocytoma and ependymoma". J Neuropathol Exp Neurol. 64 (10): 875–81. PMID 16215459.
  9. Cairncross JG, Ueki K, Zlatescu MC, Lisle DK, Finkelstein DM, Hammond RR; et al. (1998). "Specific genetic predictors of chemotherapeutic response and survival in patients with anaplastic oligodendrogliomas". J Natl Cancer Inst. 90 (19): 1473–9. PMID 9776413.
  10. Ohgaki H, Kleihues P (2005). "Population-based studies on incidence, survival rates, and genetic alterations in astrocytic and oligodendroglial gliomas". J Neuropathol Exp Neurol. 64 (6): 479–89. PMID 15977639.
  11. Hartmann C, Hentschel B, Wick W, Capper D, Felsberg J, Simon M; et al. (2010). "Patients with IDH1 wild type anaplastic astrocytomas exhibit worse prognosis than IDH1-mutated glioblastomas, and IDH1 mutation status accounts for the unfavorable prognostic effect of higher age: implications for classification of gliomas". Acta Neuropathol. 120 (6): 707–18. doi:10.1007/s00401-010-0781-z. PMID 21088844.
  12. Eckel-Passow JE, Lachance DH, Molinaro AM, Walsh KM, Decker PA, Sicotte H; et al. (2015). "Glioma Groups Based on 1p/19q, IDH, and TERT Promoter Mutations in Tumors". N Engl J Med. 372 (26): 2499–508. doi:10.1056/NEJMoa1407279. PMC 4489704. PMID 26061753.
  13. Mur P, Mollejo M, Hernández-Iglesias T, de Lope ÁR, Castresana JS, García JF; et al. (2015). "Molecular classification defines 4 prognostically distinct glioma groups irrespective of diagnosis and grade". J Neuropathol Exp Neurol. 74 (3): 241–9. doi:10.1097/NEN.0000000000000167. PMID 25668564.
  14. Reuss DE, Mamatjan Y, Schrimpf D, Capper D, Hovestadt V, Kratz A; et al. (2015). "IDH mutant diffuse and anaplastic astrocytomas have similar age at presentation and little difference in survival: a grading problem for WHO". Acta Neuropathol. 129 (6): 867–73. doi:10.1007/s00401-015-1438-8. PMC 4500039. PMID 25962792.
  15. Olar A, Wani KM, Alfaro-Munoz KD, Heathcock LE, van Thuijl HF, Gilbert MR; et al. (2015). "IDH mutation status and role of WHO grade and mitotic index in overall survival in grade II-III diffuse gliomas". Acta Neuropathol. 129 (4): 585–96. doi:10.1007/s00401-015-1398-z. PMC 4369189. PMID 25701198.
  16. Delev D, Heiland DH, Franco P, Reinacher P, Mader I, Staszewski O; et al. (2019). "Surgical management of lower-grade glioma in the spotlight of the 2016 WHO classification system". J Neurooncol. 141 (1): 223–233. doi:10.1007/s11060-018-03030-w. PMID 30467813.
  17. Iuchi T, Sugiyama T, Ohira M, Kageyama H, Yokoi S, Sakaida T; et al. (2018). "Clinical significance of the 2016 WHO classification in Japanese patients with gliomas". Brain Tumor Pathol. 35 (2): 71–80. doi:10.1007/s10014-018-0309-0. PMID 29470683.
  18. Kros JM, Gorlia T, Kouwenhoven MC, Zheng PP, Collins VP, Figarella-Branger D; et al. (2007). "Panel review of anaplastic oligodendroglioma from European Organization For Research and Treatment of Cancer Trial 26951: assessment of consensus in diagnosis, influence of 1p/19q loss, and correlations with outcome". J Neuropathol Exp Neurol. 66 (6): 545–51. doi:10.1097/01.jnen.0000263869.84188.72. PMID 17549014.
  19. van den Bent MJ (2010). "Interobserver variation of the histopathological diagnosis in clinical trials on glioma: a clinician's perspective". Acta Neuropathol. 120 (3): 297–304. doi:10.1007/s00401-010-0725-7. PMC 2910894. PMID 20644945.
  20. Reifenberger J, Reifenberger G, Liu L, James CD, Wechsler W, Collins VP (1994). "Molecular genetic analysis of oligodendroglial tumors shows preferential allelic deletions on 19q and 1p". Am J Pathol. 145 (5): 1175–90. PMC 1887413. PMID 7977648.
  21. Ueki K, Nishikawa R, Nakazato Y, Hirose T, Hirato J, Funada N; et al. (2002). "Correlation of histology and molecular genetic analysis of 1p, 19q, 10q, TP53, EGFR, CDK4, and CDKN2A in 91 astrocytic and oligodendroglial tumors". Clin Cancer Res. 8 (1): 196–201. PMID 11801559.
  22. Giannini C, Scheithauer BW, Weaver AL, Burger PC, Kros JM, Mork S; et al. (2001). "Oligodendrogliomas: reproducibility and prognostic value of histologic diagnosis and grading". J Neuropathol Exp Neurol. 60 (3): 248–62. PMID 11245209.
  23. Miller CR, Dunham CP, Scheithauer BW, Perry A (2006). "Significance of necrosis in grading of oligodendroglial neoplasms: a clinicopathologic and genetic study of newly diagnosed high-grade gliomas". J Clin Oncol. 24 (34): 5419–26. doi:10.1200/JCO.2006.08.1497. PMID 17135643.
  24. Komori T, Hirose T, Shibuya M, Suzuki H, Tanaka S, Sasaki A (2013). "Controversies over the diagnosis of oligodendroglioma: a report from the satellite workshop at the 4th international symposium of brain tumor pathology, Nagoya Congress Center, May 23, 2012". Brain Tumor Pathol. 30 (4): 253–61. doi:10.1007/s10014-013-0165-x. PMID 24100794.
  25. Takahashi K, Tsuda M, Kanno H, Murata J, Mahabir R, Ishida Y; et al. (2014). "Differential diagnosis of small cell glioblastoma and anaplastic oligodendroglioma: a case report of an elderly man". Brain Tumor Pathol. 31 (2): 118–23. doi:10.1007/s10014-013-0158-9. PMID 23979650.
  26. Sahm F, Reuss D, Koelsche C, Capper D, Schittenhelm J, Heim S; et al. (2014). "Farewell to oligoastrocytoma: in situ molecular genetics favor classification as either oligodendroglioma or astrocytoma". Acta Neuropathol. 128 (4): 551–9. doi:10.1007/s00401-014-1326-7. PMID 25143301.
  27. Yan H, Parsons DW, Jin G, McLendon R, Rasheed BA, Yuan W; et al. (2009). "IDH1 and IDH2 mutations in gliomas". N Engl J Med. 360 (8): 765–73. doi:10.1056/NEJMoa0808710. PMC 2820383. PMID 19228619.
  28. Hartmann C, Meyer J, Balss J, Capper D, Mueller W, Christians A; et al. (2009). "Type and frequency of IDH1 and IDH2 mutations are related to astrocytic and oligodendroglial differentiation and age: a study of 1,010 diffuse gliomas". Acta Neuropathol. 118 (4): 469–74. doi:10.1007/s00401-009-0561-9. PMID 19554337.
  29. Suzuki H, Aoki K, Chiba K, Sato Y, Shiozawa Y, Shiraishi Y; et al. (2015). "Mutational landscape and clonal architecture in grade II and III gliomas". Nat Genet. 47 (5): 458–68. doi:10.1038/ng.3273. PMID 25848751.
  30. Louis DN, Perry A, Burger P, Ellison DW, Reifenberger G, von Deimling A; et al. (2014). "International Society Of Neuropathology--Haarlem consensus guidelines for nervous system tumor classification and grading". Brain Pathol. 24 (5): 429–35. doi:10.1111/bpa.12171. PMID 24990071.
  31. Radner H, Blümcke I, Reifenberger G, Wiestler OD (2002). "[The new WHO classification of tumors of the nervous system 2000. Pathology and genetics]". Pathologe. 23 (4): 260–83. PMID 12185780.
  32. Leeper HE, Caron AA, Decker PA, Jenkins RB, Lachance DH, Giannini C (2015). "IDH mutation, 1p19q codeletion and ATRX loss in WHO grade II gliomas". Oncotarget. 6 (30): 30295–305. doi:10.18632/oncotarget.4497. PMC 4745799. PMID 26210286.
  33. Sabha N, Knobbe CB, Maganti M, Al Omar S, Bernstein M, Cairns R; et al. (2014). "Analysis of IDH mutation, 1p/19q deletion, and PTEN loss delineates prognosis in clinical low-grade diffuse gliomas". Neuro Oncol. 16 (7): 914–23. doi:10.1093/neuonc/not299. PMC 4057130. PMID 24470545.
  34. Yang P, Cai J, Yan W, Zhang W, Wang Y, Chen B; et al. (2016). "Classification based on mutations of TERT promoter and IDH characterizes subtypes in grade II/III gliomas". Neuro Oncol. 18 (8): 1099–108. doi:10.1093/neuonc/now021. PMC 4933482. PMID 26957363.
  35. Labussière M, Boisselier B, Mokhtari K, Di Stefano AL, Rahimian A, Rossetto M; et al. (2014). "Combined analysis of TERT, EGFR, and IDH status defines distinct prognostic glioblastoma classes". Neurology. 83 (13): 1200–6. doi:10.1212/WNL.0000000000000814. PMID 25150284.
  36. Khuong-Quang DA, Buczkowicz P, Rakopoulos P, Liu XY, Fontebasso AM, Bouffet E; et al. (2012). "K27M mutation in histone H3.3 defines clinically and biologically distinct subgroups of pediatric diffuse intrinsic pontine gliomas". Acta Neuropathol. 124 (3): 439–47. doi:10.1007/s00401-012-0998-0. PMC 3422615. PMID 22661320.
  37. Castel D, Philippe C, Calmon R, Le Dret L, Truffaux N, Boddaert N; et al. (2015). "Histone H3F3A and HIST1H3B K27M mutations define two subgroups of diffuse intrinsic pontine gliomas with different prognosis and phenotypes". Acta Neuropathol. 130 (6): 815–27. doi:10.1007/s00401-015-1478-0. PMC 4654747. PMID 26399631.
  38. Castel D, Grill J, Debily MA (2016). "Histone H3 genotyping refines clinico-radiological diagnostic and prognostic criteria in DIPG". Acta Neuropathol. 131 (5): 795–6. doi:10.1007/s00401-016-1568-7. PMC 4835508. PMID 27038188.
  39. Jiao Y, Killela PJ, Reitman ZJ, Rasheed AB, Heaphy CM, de Wilde RF; et al. (2012). "Frequent ATRX, CIC, FUBP1 and IDH1 mutations refine the classification of malignant gliomas". Oncotarget. 3 (7): 709–22. doi:10.18632/oncotarget.588. PMC 3443254. PMID 22869205.
  40. Yip S, Butterfield YS, Morozova O, Chittaranjan S, Blough MD, An J; et al. (2012). "Concurrent CIC mutations, IDH mutations, and 1p/19q loss distinguish oligodendrogliomas from other cancers". J Pathol. 226 (1): 7–16. doi:10.1002/path.2995. PMC 3246739. PMID 22072542.
  41. Frenel JS, Leux C, Loussouarn D, Le Loupp AG, Leclair F, Aumont M; et al. (2013). "Combining two biomarkers, IDH1/2 mutations and 1p/19q codeletion, to stratify anaplastic oligodendroglioma in three groups: a single-center experience". J Neurooncol. 114 (1): 85–91. doi:10.1007/s11060-013-1152-0. PMID 23681562.
  42. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A; et al. (2007). "The 2007 WHO classification of tumours of the central nervous system". Acta Neuropathol. 114 (2): 97–109. doi:10.1007/s00401-007-0243-4. PMC 1929165. PMID 17618441.