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

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 locations 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)

They are generally composed of cells with small to slightly enlarged round nuclei with dark, compact nuclei and a small amount of eosinophilic cytoplasm. They are often referred to as "fried egg" cells due to their histologic appearance. They appear as a monotonous population of mildly enlarged round cells infiltrating normal brain parenchyma and producing vague nodules. Although the tumor may appear to be vaguely circumscribed, it is by definition a diffusely infiltrating tumor.

Classically they tend to have a vasculature of finely branching capillaries that may take on a “chicken wire” appearance . When invading grey matter structures such as cortex, the neoplastic oligodendrocytes tend to cluster around neurons exhibiting a phenomenon referred to as “perineuronal satellitosis”. Oligodendrogliomas may invade preferentially around vessels or under the pial surface of the brain.

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