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oligodendroglioma (oligodendroblastoma)

WHO grade 2 glioma. Epidemiology: - generally occurs in adults Pathology: 1) preferentially affects white matter & basal ganglia (supratentorial) 2) generally less infiltrative than astrocytic neoplasms Microscopic Pathology: 1) tumors are moderately cellular with round hyperchromatic nuclei & artifactual perinuclear clearing 2) 30% are calcified 3) may contain mixtures of astrocytic & oligodenroglial forms 4) features of mitoses, necrosis & nuclear atypia are associated with a more aggressive clinical course Immunopathology: - topoisomerase II-alpha: high levels correlated with high tumor grade, high proliferation rate, p53 overexpression, & high expression of Rb protein [4] Genetics: 1) chromosomal deletions: - 1p, 19q deletions associated with response to PCV 2) associated with defects in CAMTA1 gene 3) other implicated genes TP73, PTEN, IDH1 (R132H mutation) [6] * also see ARUP consult [7] Clinical manifestations: - case presentation of woman with grand mal seizures 4 days after C-section [6] Radiology: - magnetic resonance imaging: - infiltrating white matter lesion - vague contrast enhancement - honeycomb pattern - 90% of oligodendrogliomas are calcified - intracranial calcification best seen on computed tomography (CT) Management: 1) surgical resection -> many tumors amenable to total resection 2) chemotherapy a lomustine/procarbazine/vincristine (PCV) b) carmustine c) temozolomide [5] 3) radiation therapy 4) stereotaxic radiosurgery with gamma knife 5) prognosis a) 5 year survival is > 50% b) 10 year survival is 25-34%

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oligodendrocyte

General

oligodendroglial neoplasm

References

  1. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 2398
  2. Harrison's Online, Chapter 370, McGraw-Hill, 2002
  3. WHO Classification Tumours of the Nervous System. Kleihues & Cavenee eds. IARC Press 2000
  4. Miettinen HE et al High topoisomerase IIalpha expression associates with high proliferation rate and and poor prognosis in oligodendrogliomas. Neuropathology & Applied Neurobiology 26:504-12, 2000 PMID: 11123716
  5. Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 18. American College of Physicians, Philadelphia 2009, 2012, 2018
  6. Chi AS, Cahill DP, Larvie M, Louis DN Case 38-2016 - A 52-Year-Old Woman with Recurrent Oligodendroglioma. N Engl J Med 2016; 375:2381-2389. December 15, 2016 PMID: 27974037 http://www.nejm.org/doi/full/10.1056/NEJMcpc1610101
  7. ARUP Consult: 1p/19q Deletion in Oligodendrogliomas https://arupconsult.com/ati/1p19q-deletion-oligodendrogliomas

Databases & Images

OMIM 137800 images related to oligodendroglioma