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Clinical features, pathology, and prognostic factors for oligodendroglial tumors

Martin van den Bent, MD, PhD
Section Editors
Patrick Y Wen, MD
Jay S Loeffler, MD
Deputy Editor
April F Eichler, MD, MPH


There are significant differences between oligodendrogliomas and oligoastrocytomas and other glial tumors in pathology, molecular pathogenesis, and natural history. These differences have important prognostic implications, which can affect treatment.

The clinical manifestations, classification, pathologic classification, and molecular prognostic factors associated with oligodendroglial tumors will be reviewed here. The treatment of these tumors is discussed separately. (See "Management of anaplastic oligodendroglial tumors".)


Clinical features — The presenting signs and symptoms of oligodendroglial tumors are nonspecific and depend upon the location and extent of the tumor. Symptoms may be either generalized (eg, headaches, seizures, cognitive dysfunction) or focal (eg, weakness, sensory abnormalities, aphasia). There are no clinical features that distinguish these tumors from other gliomas. (See "Clinical presentation and diagnosis of brain tumors".)

Most oligodendroglial tumors arise in the white matter of the cerebral hemispheres, predominantly in the frontal lobes [1]. However, they can also arise at infratentorial sites and in the spinal cord. As with other glial tumors, oligodendrogliomas and oligoastrocytomas only rarely metastasize outside the central nervous system.

Epidemiology — Oligodendroglial tumors constitute between 5 and 20 percent of all glial tumors. The frequency of these lesions is higher in contemporary reports due primarily to modifications in subjective histopathologic criteria, but more recently the presence of more classical histopathologic features for this diagnosis is emphasized [2].


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Literature review current through: Sep 2016. | This topic last updated: Oct 30, 2015.
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  1. Lassman AB, Iwamoto FM, Cloughesy TF, et al. International retrospective study of over 1000 adults with anaplastic oligodendroglial tumors. Neuro Oncol 2011; 13:649.
  2. Claus EB, Black PM. Survival rates and patterns of care for patients diagnosed with supratentorial low-grade gliomas: data from the SEER program, 1973-2001. Cancer 2006; 106:1358.
  3. Lee YY, Van Tassel P. Intracranial oligodendrogliomas: imaging findings in 35 untreated cases. AJR Am J Roentgenol 1989; 152:361.
  4. Jenkinson MD, du Plessis DG, Smith TS, et al. Histological growth patterns and genotype in oligodendroglial tumours: correlation with MRI features. Brain 2006; 129:1884.
  5. Megyesi JF, Kachur E, Lee DH, et al. Imaging correlates of molecular signatures in oligodendrogliomas. Clin Cancer Res 2004; 10:4303.
  6. Reifenberger G, Kros JM, Louis DN, Collins VN. Oligodendroglioma. In: World Health Organization Classification of Tumours, Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (Eds), IARC Press, Lyon 2007. p.53.
  7. Krouwer HG, van Duinen SG, Kamphorst W, et al. Oligoastrocytomas: a clinicopathological study of 52 cases. J Neurooncol 1997; 33:223.
  8. Sahm F, Reuss D, Koelsche C, et al. Farewell to oligoastrocytoma: in situ molecular genetics favor classification as either oligodendroglioma or astrocytoma. Acta Neuropathol 2014; 128:551.
  9. Miller CR, Dunham CP, Scheithauer BW, Perry A. Significance of necrosis in grading of oligodendroglial neoplasms: a clinicopathologic and genetic study of newly diagnosed high-grade gliomas. J Clin Oncol 2006; 24:5419.
  10. Wang Y, Li S, Chen L, et al. Glioblastoma with an oligodendroglioma component: distinct clinical behavior, genetic alterations, and outcome. Neuro Oncol 2012; 14:518.
  11. Dubbink HJ, Atmodimedjo PN, Kros JM, et al. Molecular classification of anaplastic oligodendroglioma using next-generation sequencing: a report of the prospective randomized EORTC Brain Tumor Group 26951 phase III trial. Neuro Oncol 2016; 18:388.
  12. Reuss DE, Sahm F, Schrimpf D, et al. 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. Acta Neuropathol 2015; 129:133.
  13. Fallon KB, Palmer CA, Roth KA, et al. Prognostic value of 1p, 19q, 9p, 10q, and EGFR-FISH analyses in recurrent oligodendrogliomas. J Neuropathol Exp Neurol 2004; 63:314.
  14. Griffin CA, Burger P, Morsberger L, et al. Identification of der(1;19)(q10;p10) in five oligodendrogliomas suggests mechanism of concurrent 1p and 19q loss. J Neuropathol Exp Neurol 2006; 65:988.
  15. Jenkins RB, Blair H, Ballman KV, et al. A t(1;19)(q10;p10) mediates the combined deletions of 1p and 19q and predicts a better prognosis of patients with oligodendroglioma. Cancer Res 2006; 66:9852.
  16. Bigner SH, Matthews MR, Rasheed BK, et al. Molecular genetic aspects of oligodendrogliomas including analysis by comparative genomic hybridization. Am J Pathol 1999; 155:375.
  17. Zlatescu MC, TehraniYazdi A, Sasaki H, et al. Tumor location and growth pattern correlate with genetic signature in oligodendroglial neoplasms. Cancer Res 2001; 61:6713.
  18. Mueller W, Hartmann C, Hoffmann A, et al. Genetic signature of oligoastrocytomas correlates with tumor location and denotes distinct molecular subsets. Am J Pathol 2002; 161:313.
  19. Watanabe T, Nobusawa S, Kleihues P, Ohgaki H. IDH1 mutations are early events in the development of astrocytomas and oligodendrogliomas. Am J Pathol 2009; 174:1149.
  20. Gorlia T, Delattre JY, Brandes AA, et al. New clinical, pathological and molecular prognostic models and calculators in patients with locally diagnosed anaplastic oligodendroglioma or oligoastrocytoma. A prognostic factor analysis of European Organisation for Research and Treatment of Cancer Brain Tumour Group Study 26951. Eur J Cancer 2013; 49:3477.
  21. Panageas KS, Reiner AS, Iwamoto FM, et al. Recursive partitioning analysis of prognostic variables in newly diagnosed anaplastic oligodendroglial tumors. Neuro Oncol 2014; 16:1541.
  22. Leighton C, Fisher B, Bauman G, et al. Supratentorial low-grade glioma in adults: an analysis of prognostic factors and timing of radiation. J Clin Oncol 1997; 15:1294.
  23. Shaw EG, Scheithauer BW, O'Fallon JR, et al. Oligodendrogliomas: the Mayo Clinic experience. J Neurosurg 1992; 76:428.
  24. Shaw EG, Scheithauer BW, O'Fallon JR. Supratentorial gliomas: a comparative study by grade and histologic type. J Neurooncol 1997; 31:273.
  25. Kouwenhoven MC, Gorlia T, Kros JM, et al. Molecular analysis of anaplastic oligodendroglial tumors in a prospective randomized study: A report from EORTC study 26951. Neuro Oncol 2009; 11:737.
  26. Intergroup Radiation Therapy Oncology Group Trial 9402, Cairncross G, Berkey B, et al. Phase III trial of chemotherapy plus radiotherapy compared with radiotherapy alone for pure and mixed anaplastic oligodendroglioma: Intergroup Radiation Therapy Oncology Group Trial 9402. J Clin Oncol 2006; 24:2707.
  27. van den Bent MJ, Carpentier AF, Brandes AA, et al. Adjuvant procarbazine, lomustine, and vincristine improves progression-free survival but not overall survival in newly diagnosed anaplastic oligodendrogliomas and oligoastrocytomas: a randomized European Organisation for Research and Treatment of Cancer phase III trial. J Clin Oncol 2006; 24:2715.
  28. van DEN Bent. Long-term follow-up results of EORTC 26951: A randomized phase III study on adjuvant PCV chemotherapy in anaplastic oligodendroglial tumors (AOD) (abstract #2). J Clin Oncol 2012; 30.
  29. Cairncross JG, Wang M, Shaw EG, et al. Chemotherapy plus radiotherapy versus RT alone for patients with anaplastic oligodendroglioma: Long-term results of the RTOG 9402 phase III study (abstract #2008b). J Clin Oncol 2012; 30s:2008b.
  30. Wick W, Hartmann C, Engel C, et al. NOA-04 randomized phase III trial of sequential radiochemotherapy of anaplastic glioma with procarbazine, lomustine, and vincristine or temozolomide. J Clin Oncol 2009; 27:5874.
  31. Wick W, Meisner C, Hentschel B, et al. Prognostic or predictive value of MGMT promoter methylation in gliomas depends on IDH1 mutation. Neurology 2013; 81:1515.
  32. Snuderl M, Eichler AF, Ligon KL, et al. Polysomy for chromosomes 1 and 19 predicts earlier recurrence in anaplastic oligodendrogliomas with concurrent 1p/19q loss. Clin Cancer Res 2009; 15:6430.
  33. Jiang H, Ren X, Zhang Z, et al. Polysomy of chromosomes 1 and 19: an underestimated prognostic factor in oligodendroglial tumors. J Neurooncol 2014; 120:131.
  34. Gleize V, Alentorn A, Connen de Kérillis L, et al. CIC inactivating mutations identify aggressive subset of 1p19q codeleted gliomas. Ann Neurol 2015; 78:355.
  35. van den Bent MJ, Dubbink HJ, Marie Y, et al. IDH1 and IDH2 mutations are prognostic but not predictive for outcome in anaplastic oligodendroglial tumors: a report of the European Organization for Research and Treatment of Cancer Brain Tumor Group. Clin Cancer Res 2010; 16:1597.
  36. Cairncross JG, Wang M, Jenkins RB, et al. Benefit from procarbazine, lomustine, and vincristine in oligodendroglial tumors is associated with mutation of IDH. J Clin Oncol 2014; 32:783.
  37. Watanabe T, Nakamura M, Kros JM, et al. Phenotype versus genotype correlation in oligodendrogliomas and low-grade diffuse astrocytomas. Acta Neuropathol 2002; 103:267.
  38. Nutt CL, Costello JF, Bambrick LL, et al. O6-methylguanine-DNA methyltransferase in tumors and cells of the oligodendrocyte lineage. Can J Neurol Sci 1995; 22:111.
  39. van den Bent MJ, Dubbink HJ, Sanson M, et al. MGMT promoter methylation is prognostic but not predictive for outcome to adjuvant PCV chemotherapy in anaplastic oligodendroglial tumors: a report from EORTC Brain Tumor Group Study 26951. J Clin Oncol 2009; 27:5881.
  40. Dong SM, Pang JC, Poon WS, et al. Concurrent hypermethylation of multiple genes is associated with grade of oligodendroglial tumors. J Neuropathol Exp Neurol 2001; 60:808.
  41. Möllemann M, Wolter M, Felsberg J, et al. Frequent promoter hypermethylation and low expression of the MGMT gene in oligodendroglial tumors. Int J Cancer 2005; 113:379.
  42. Brandes AA, Tosoni A, Cavallo G, et al. Correlations between O6-methylguanine DNA methyltransferase promoter methylation status, 1p and 19q deletions, and response to temozolomide in anaplastic and recurrent oligodendroglioma: a prospective GICNO study. J Clin Oncol 2006; 24:4746.
  43. van den Bent MJ, Erdem-Eraslan L, Idbaih A, et al. MGMT-STP27 methylation status as predictive marker for response to PCV in anaplastic Oligodendrogliomas and Oligoastrocytomas. A report from EORTC study 26951. Clin Cancer Res 2013; 19:5513.
  44. Ramirez C, Bowman C, Maurage CA, et al. Loss of 1p, 19q, and 10q heterozygosity prospectively predicts prognosis of oligodendroglial tumors--towards individualized tumor treatment? Neuro Oncol 2010; 12:490.
  45. Cairncross G, Macdonald D, Ludwin S, et al. Chemotherapy for anaplastic oligodendroglioma. National Cancer Institute of Canada Clinical Trials Group. J Clin Oncol 1994; 12:2013.