Oligodendroglioma pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [4]Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[5]Sujit Routray, M.D. [6]

Overview

Oligodendroglioma arises from the tripotential glial precursor cells and not from the bipotential oligodendrocytes. Genes associated with the pathogenesis of oligodendroglioma include t[1;19][q10;p10], ATRX, NJDS, IDH1, IDH2, TERT promoter, H3 K27M (H3F3A, HIST1H3B/C), CIC, FUBP1, p53, Leu-7, TCF-12, TP53,MGMT, TP73, BRAF, EGFR, and PTEN. Common intracranial sites involved by oligodendroglioma include cerebral hemispheres, posterior fossa, and intramedullary spinal cord. On gross pathology, oligodendroglioma is characterized by a well-circumscribed, gelatinous, calcified, cystic, gray mass with focal hemorrhage which may expand a gyrus and remodel the skull. On microscopic histopathological analysis, oligodendroglioma is characterized by diffuse growthpattern of highly cellular lesion of monomorphic cells having rounded nucleus with atypia, speckled "salt-and-pepper" chromatin pattern and perinuclear haloresembling fried eggs, distinct cell borders, clear cytoplasm, abundant calcification and "chicken-wire" like vascularity pattern. Oligodendroglioma is demonstrated by positivity to tumor markers such as IDH1-R132H, MAP2, GFAP, S-100, SOX10, EMA, ATRX, Ki-67, NSE, synaptophysin, OLIG1, and OLIG2.

Pathophysiology

Pathogenesis

Genetics

Gross Pathology

Microscopic Pathology

On microscopic histopathological analysis, oligodendroglioma is characterized by:[20][60][61][62][63]

  • Diffusely growing, infiltrative tumor
  • Moderate cellularity
  • Highly cellular lesion composed of typically monomorphic cells resembling fried eggs with:
    • Round nucleus - key feature
    • Distinct cell borders
    • Moderate-to-marked nuclear atypia with speckled "salt-and-pepper" chromatin pattern
    • Inconspicuous nucleoli
    • Clear cytoplasm (artifactual retraction of the cytoplasm on routinely processed formalin fixed, paraffin embedded material, leading to the characteristic "fried egg" appearance)
      • Some oligodendrogliomas have eosinophilic cytoplasm with focal perinuclear clearing
    • Dense network of acutely fine branched capillary sized vessels -classically referred to as a "chicken-wire" like appearance/pattern[64]
      • Abundant and delicate appearing; may vaguely resemble a paraganglioma at low power
  • Small punctate calcifications, particularly along the blood vessels is a striking feature (but not a specific finding)
  • Perifocal edema - rare
  • Few tumors may exhibit eosinophilic granular bodies
  • Some tumors may show a spongioblastoma-like growth pattern
  • Tumor cells may form following secondary structures in the surrounding infiltrated brain parenchyma:
    • Perineuronal satellitosis
    • Subpial accumulation
    • Perivascular distribution (less common)
  • Microgemistocytic appearance of tumor cells with a rounded belly of eccentric GFAP+ eosinophilic cytoplasm (maybe present)[65]
  • A predominant fibrillar astrocytic phenotype is compatible with the diagnosis when following appropriate molecular findings are present:[21]
    • IDH mutation
    • 1p/19q codeletion

Microscopic histopathological findings in anaplastic oligodendroglioma

On microscopic histopathological analysis, anaplastic oligodendroglioma, IDH mutant and 1p/19q codeleted, is characterized by:[60]

Brisk mitotic rate in anaplastic oligodendroglioma [1]
High magnification micrograph of an oligodendroglioma showing the characteristic branching, small, chicken wire-like blood vessels and fried egg-like cells, with clear cytoplasm and well-defined cell borders. H&E stain. [2]
Low magnification micrograph of an oligodendroglioma showing the characteristic, small, branching, chicken wire-like blood vessels. H&E stain. [3]
"Fried egg" appearance Source: John DeWitt, M.D., Ph.D.
Chicken wire vessels Source: John DeWitt, M.D., Ph.D.
"Fried egg" appearance Source: John DeWitt, M.D., Ph.D.
Vascularproliferation Source: Roger E McLendon, MD et al.
Infiltrating cortex Source: John DeWitt, M.D., Ph.D.

Immunohistochemistry

Oligodendroglioma is demonstrated by positivity to tumor markers such as:[68][69][20][7]

Oligodendroglioma stains negative for:

References

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