eMedicine Specialties > Neurology > Neuro-oncology

Oligodendroglioma

Author: ABM Salah Uddin, MD, Consulting Staff, Department of Internal Medicine, St Vincent's Hospital
Coauthor(s): Tambi Jarmi, MD, Resident Physician, Department of Internal Medicine, Carraway Methodist Medical Center
Contributor Information and Disclosures

Updated: May 4, 2009

Introduction

Background

Oligodendrogliomas (ODs) are primary glial brain tumors that are divided into grade II and anaplastic grade III tumors (World Health Organization [WHO] criteria). Typically, they have an indolent course, and patients may survive for many years after symptom onset. Their good prognosis relative to other parenchymal tumors probably stems from inherently less aggressive biological behavior and a favorable response to chemotherapy, a recently discovered finding based on genetic characteristics.

Pathophysiology

Oligodendrogliomas arise in the cerebral hemispheres and are distributed among the frontal, parietal, temporal, and occipital lobe, in approximately a 3:2:2:1 ratio. Rarely, they can arise in the cerebellum, brain stem, and spinal cord. They usually occur in the cerebral white matter and are very cellular, with uniform nuclei. They react with glial fibrillary acidic protein on immunostaining.

Frequency

United States

The incidence of oligodendrogliomas ranges from 5-19% of all intracranial tumors. The newer studies showed incidence of oligodendrogliomas to be around 25% of all gliomas. This may be explained by the improvements in the treatment of oligodendrogliomas, prompting neuropathologists to favor the diagnosis.

International

No difference in the incidence of oligodendroglioma exists worldwide.

Mortality/Morbidity

The morbidity and mortality profile for oligodendrogliomas is much better than for astrocytic tumors. However, it also depends on tumor location and pressure effects, as with any other intracranial lesion. The median survival from initial diagnosis of all low-grade oligodendrogliomas (LGOs) is 4-10 years, but it is only 3-4 years for anaplastic oligodendrogliomas.

Race

No difference exists among the races.

Sex

Oligodendrogliomas occur in both sexes, with a male-to-female predominance of 2:1.

Age

Oligodendrogliomas may be diagnosed at any age but occur most commonly in young and middle-aged adults, with a median age at diagnosis of 40-50 years. In children, only 6% of gliomas are diagnosed as oligodendrogliomas.

Clinical

History

  • In prior years, a long delay occurred between symptom onset and diagnosis (as long as 29 y in some series). Because of earlier and better imaging availability, oligodendrogliomas have been diagnosed much earlier in recent years.
  • Like other intracranial space-occupying lesions, oligodendrogliomas present with focal cerebral dysfunction, depending on location, and rarely as increased intracranial pressure.
    • Most oligodendrogliomas present as a single lesion in the cerebral hemispheres.
    • Typically, they are cortical or subcortical; they rarely are found in deep gray structures, and occasionally they may be primarily intraventricular.
  • Rarely, they can occur infratentorially or in the spinal cord.
  • Occasionally they may be multifocal, like other gliomas.
  • The most common presenting symptom is seizure, observed at diagnosis in as many as half of patients. As many as 80% of patients have seizures at some time during their illness.
    • Depending on the location of the tumor, the seizure can be simple partial, complex partial, or generalized.
    • Previously undiagnosed oligodendrogliomas may be identified with medically refractory epilepsy.
  • Occasionally patients with oligodendrogliomas are brought to medical attention for headache, symptoms of increased intracranial pressure, or focal neurological deficits.
  • Tumors that arise within the ventricles may cause obstructive hydrocephalus and are more likely to disseminate through the cerebrospinal fluid (CSF). Rarely, they can metastasize outside the nervous system, especially the anaplastic oligodendroglioma.
  • In long-surviving patients with 1p/19q co-deletion, indolent leptomeningeal disease may be a complication of oligodendroglioma, which may have implications for the treatment.1
  • Occasional patients present with strokelike transient ischemic attacks or with intracerebral hemorrhage.

Physical

Physical findings depend on the location of the tumor.

  • Frontal, parietal, and temporal lobe tumors most commonly present with seizures. Seizures may be simple, complex partial, and even generalized.
    • Frontoparietal tumors may present with hemiparesis and sensory neglect.
    • Sensory neglect is pronounced in right hemispheric lesions.
    • Temporal lobe tumors rarely may present with visual field defects, although patients may be unaware of hemianopsia.
  • Rare intraventricular oligodendroglioma may present with signs and symptoms of increased intracranial pressure such as headache, visual disturbance, and papilledema.
  • Posterior fossa oligodendrogliomas are uncommon. However, well-documented cases are described in children and may present with cerebellar ataxia and increased intracranial pressure.

Causes

No causes or risk factors are known. Occasional clustering occurs in some families, although the mode of inheritance is unknown. Patients with anaplastic oligodendrogliomas who have loss of heterozygosity on 1p or combined loss of heterozygosity on 1p and 19q survive substantially longer (mean, 10 y) than patients whose tumors lack these genetic changes (mean, 2 y).

More on Oligodendroglioma

Overview: Oligodendroglioma
Differential Diagnoses & Workup: Oligodendroglioma
Treatment & Medication: Oligodendroglioma
Follow-up: Oligodendroglioma
Multimedia: Oligodendroglioma
References
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References

  1. Roldan G, Scott J, George D, Parney I, Easaw J, Cairncross G. Leptomeningeal disease from oligodendroglioma: clinical and molecular analysis. Can J Neurol Sci. May 2008;35(2):204-9. [Medline].

  2. Megyesi JF, Kachur E, Lee DH, et al. Imaging correlates of molecular signatures in oligodendrogliomas. Clin Cancer Res. Jul 1 2004;10(13):4303-6. [Medline].

  3. Brown R, Zlatescu M, Sijben A, Roldan G, Easaw J, Forsyth P. The use of magnetic resonance imaging to noninvasively detect genetic signatures in oligodendroglioma. Clin Cancer Res. Apr 15 2008;14(8):2357-62. [Medline].

  4. Yuen ST, Fung CF, Ng TH, Leung SY. Central neurocytoma: its differentiation from intraventricular oligodendroglioma. Childs Nerv Syst. Oct 1992;8(7):383-8. [Medline].

  5. Burger PC, Rawlings CE, Cox EB, et al. Clinicopathologic correlations in the oligodendroglioma. Cancer. Apr 1 1987;59(7):1345-52. [Medline].

  6. van den Bent MJ. Advances in the biology and treatment of oligodendrogliomas. Curr Opin Neurol. Dec 2004;17(6):675-80. [Medline].

  7. [Best Evidence] van den Bent MJ, Carpentier AF, Brandes AA, Sanson M, Taphoorn MJ, Bernsen HJ, 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. Jun 20 2006;24(18):2715-22. [Medline].

  8. Cairncross G, Macdonald D, Ludwin S, et al. Chemotherapy for anaplastic oligodendroglioma. National Cancer Institute of Canada Clinical Trials Group. J Clin Oncol. Oct 1994;12(10):2013-21. [Medline].

  9. van den Bent MJ, Taphoorn MJ, Brandes AA, et al. Phase II study of first-line chemotherapy with temozolomide in recurrent oligodendroglial tumors: the European Organization for Research and Treatment of Cancer Brain Tumor Group Study 26971. J Clin Oncol. Jul 1 2003;21(13):2525-8. [Medline]. [Full Text].

  10. Mohile NA, Forsyth P, Stewart D, Raizer JJ, Paleologos N, Kewalramani T, et al. A phase II study of intensified chemotherapy alone as initial treatment for newly diagnosed anaplastic oligodendroglioma: an interim analysis. J Neurooncol. Sep 2008;89(2):187-93. [Medline].

  11. Cairncross JG, Berkey B, Shaw E. Phase III trial of chemotherapy plus radiotherapy compared with radiotherapy alone for pure and mixed anaplastic oligodendrogliomas: intergroup Radiation Therapy Oncology Group Trial 9402. J Clin Oncol. 2006;24:2702-2714.

  12. Daumas-Duport C, Scheithauer BW, Chodkiewicz JP, et al. Dysembryoplastic neuroepithelial tumor: a surgically curable tumor of young patients with intractable partial seizures. Report of thirty-nine cases. Neurosurgery. Nov 1988;23(5):545-56. [Medline].

  13. Bello MJ, Vaquero J, de Campos JM, et al. Molecular analysis of chromosome 1 abnormalities in human gliomas reveals frequent loss of 1p in oligodendroglial tumors. Int J Cancer. Apr 15 1994;57(2):172-5. [Medline].

  14. Celli P, Nofrone I, Palma L, et al. Cerebral oligodendroglioma: prognostic factors and life history. Neurosurgery. Dec 1994;35(6):1018-34; discussion 1034-5. [Medline].

  15. Han SR, Yoon SW, Yee GT, Choi CY, Lee DJ, Sohn MJ, et al. Extraneural metastases of anaplastic oligodendroglioma. J Clin Neurosci. Aug 2008;15(8):946-9. [Medline].

  16. Hartmann C, von Deimling A. Oligodendrogliomas: impact of molecular genetics on treatment. Neurol India. Jun 2005;53(2):140-8. [Medline].

  17. Kang SG, Kim JH, Nam do H, Park K. Clinical and radiological prognostic factors of anaplastic oligodendroglioma treated by combined therapy. Neurol Med Chir (Tokyo). May 2005;45(5):232-8; discussion 238-9. [Medline].

  18. Kaye AH, Laws ER Jr, eds. Brain Tumors: An Encyclopedic Approach. New York: Churchill Livingstone; 1995:479-91.

  19. Kleihus P, Cavenee WK. Pathology and Genetics of Tumours of the Nervous System. New York: Oxford University Press; 2000:56-64.

  20. Mason WP. Oligodendroglioma. Curr Treat Options Neurol. Jul 2005;7(4):305-314. [Medline].

  21. Mason WP, DeAngelis LM. Procarbazine, CCNU, vincristine (PCV) chemotherapy for benign oligodendroglioma. Neurology. 1994;44(Suppl 2):A262-A263.

  22. Packer RJ, Sutton LN, Rorke LB, et al. Oligodendroglioma of the posterior fossa in childhood. Cancer. Jul 1 1985;56(1):195-9. [Medline].

  23. Paleologos NA, Vick NA, Kachoris JP. Chemotherapy for low grade oligodendrogliomas. Ann Neurol. 1994;36:294-295.

  24. Pitt MA, Jones AW, Reeve RS, Cowie RA. Oligodendroglioma of the fourth ventricle with intracranial and spinal oligodendrogliomatosis: a case report. Br J Neurosurg. 1992;6(4):371-4. [Medline].

  25. Sarkar C, Roy S, Tandon PN. Oligodendroglial tumors. An immunohistochemical and electron microscopic study. Cancer. May 1 1988;61(9):1862-6. [Medline].

  26. Schold SC, Burger PC, Minna JD, et al. Primary Tumors of the Brain and Spinal Cord. Boston: Butterworth Heinemann; 1997:71-82.

  27. van den Bent MJ, Afra D, de Witte O, et al. Long-term efficacy of early versus delayed radiotherapy for low-grade astrocytoma and oligodendroglioma in adults: the EORTC 22845 randomised trial. Lancet. Sep 17-23 2005;366(9490):985-90. [Medline].

  28. Wen PY, Black PM. Brain Tumors in Adults. Neurol Clin. 1995;13:861-873.

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Further Reading

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Keywords

oligodendroglioma, OD, LGO, anaplastic oligodendroglioma, glial brain tumor, intracranial tumors, low-grade oligodendrogliomas

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Contributor Information and Disclosures

Author

ABM Salah Uddin, MD, Consulting Staff, Department of Internal Medicine, St Vincent's Hospital
ABM Salah Uddin, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Tambi Jarmi, MD, Resident Physician, Department of Internal Medicine, Carraway Methodist Medical Center
Tambi Jarmi, MD is a member of the following medical societies: American College of Physicians and American Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Amy A Pruitt, MD, Associate Professor of Neurology, University of Pennsylvania; Attending Neurologist, Hospital of the University of Pennsylvania
Amy A Pruitt, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Jorge Kattah, MD, Head, Program Director, Professor, Department of Neurology, University of Illinois College of Medicine at Peoria
Jorge Kattah, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, and New York Academy of Sciences
Disclosure: Biogen Honoraria Consulting; Bayer Corporation Honoraria Consulting

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Stephen A Berman, MD, PhD, Professor, Department of Internal Medicine, Section of Neurology, Dartmouth Medical School; Chief, Neurology Service, White River Junction Veterans Medical Center
Stephen A Berman, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, and Phi Beta Kappa
Disclosure: Nothing to disclose.

 
 
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