Oligodendroglioma pathophysiology

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

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Pathophysiology

Pathogenesis

• Oligodendroglioma does not arise from the bipotential oligodendrocytes, although tumor cells look very similiar.
• Oligodendroglioma arises from the tripotential glial precursor cells.

Genetics

• Development of oligodendroglioma is the result from multiple genetic mutations.
• Genes associated with the pathogenesis of oligodendroglioma include:

• t[1;19][q10;p10] (co-deletion of chromosomal arms 1p and 19q; most common)
• NJDS
• IDH1
• IDH2
• CIC
• FUBP1
• p53
• Leu-7

Gross Pathology

• On gross pathology, oligodendroglioma is characterized by a well-circumscribed, gelatinous, gray mass which may expand a gyrus and remodel the skull.
• Other characteristic gross pathological features associated with oligodendroglioma include:

• Calcification (70-90%; one of the most frequently calcifying tumors)
• Focal hemorrhage
• Cystic (20%)

• Common intracranial sites associated with oligodendroglioma include:

• Cerebral hemispheres - most commonly frontal lobe (50-60%), followed by parietal and temporal lobes
• Posterior fossa (rare)
• Intramedullary spinal cord (very rare)

Microscopic Pathology

On microscopic histopathological analysis, oligodendroglioma is characterized by:

• Diffusely growing tumor
• Highly cellular lesion composed of cells resembling fried eggs with:
  • Round nucleus - key feature
  • Distinct cell borders
  • Moderate-to-marked nuclear atypia
  • Clear cytoplasm
    • Some oligodendrogliomas have eosinophilic cytoplasm with focal perinuclear clearing
  • Acutely branched capillary sized vessels - "chicken-wire" like appearance
    • Abundant, delicate appearing; may vaguely resemble a paraganglioma at low power
• Calcifications - striking feature
• Perifocal edema - rare
• Few tumors may exhibit eosinophilic granular bodies
• Some tumors may show a spongioblastoma-like growth pattern

On microscopic histopathological analysis, anaplastic oligodendroglioma is characterized by:

• Significant or brisk mitotic activity (>= 6 mitoses per 10 HPF)
• Microvacular proliferation
• Necrosis
• Tumor cells may be plasmacytoid (i.e. have a plasma cell-like appearance)
  • Also called as minigemistocytes

Immunohistochemistry

Oligodendroglioma is demonstrated by positivity to tumor markers such as:

• MAP2
• GFAP
• S-100
• EMA
• IDH1-R132H
• ATRX
• Ki-67
• NSE
• Synaptophysin

Gallery

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  Oligodendroglioma low magnification showing the characteristic small, branching, chicken wire-like blood vessels.H&E stain.

• 

  Oligodendroglioma high magnification showing highly cellular lesion composed of cells resembling fried eggs with distinct cell borders moderate-to-marked nuclear atypia, and a clear cytoplasm. Acutely branched capillary sized vessels - "chicken-wire" like appearance.

• 

  Low power magnification of oligodendroglioma biopsy specimen showing discrete infiltration of the surrounding brain (HE stain, x40 mag).

• 

  Histopathology of anaplastic oligodendroglioma showing minigemistocytes and mitoses among tumor cells with perinuclear halo. HE stain.

• 

  Histopathology of anaplastic oligodendroglioma (MAP2 staining) showing perinuclear immunoreactivity of tumor cells.

• 

  Histopathology of anaplastic oligodendroglioma (IDH1-R132H staining) showing immunoreactivity of tumor cells indicating presence of the isocitrate dehydrogenase 1-R132H mutation.

References

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