oligodendroglioma (oligodendroblastoma)

Introduction

WHO grade 2 glioma.

Epidemiology

• generally occurs in adults

Pathology

• preferentially affects white matter & basal ganglia (supratentorial)
• generally less infiltrative than astrocytic neoplasms

Microscopic pathology

• tumors are moderately cellular with round hyperchromatic nuclei & artifactual perinuclear clearing
• 30% are calcified
• may contain mixtures of astrocytic & oligodenroglial forms
• features of mitoses, necrosis & nuclear atypia are associated with a more aggressive clinical course

Immunophenotype

• topoisomerase II-alpha: high levels correlated with high tumor grade, high proliferation rate, p53 overexpression, & high expression of Rb protein

Genetics

• chromosomal deletions:
  • 1p, 19q deletions associated with response to PCV
• associated with defects in CAMTA1 gene
• other implicated genes TP73, PTEN, IDH1 (R132H mutation)

* also see ARUP consult

Clinical manifestations

• case presentation of woman with grand mal seizures 4 days after C-section

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

• surgical resection
  • many tumors amenable to total resection
• chemotherapy a lomustine/procarbazine/vincristine (PCV)
  • carmustine
  • temozolomide
• radiation therapy
• stereotaxic radiosurgery with gamma knife
• prognosis
  • 5 year survival is > 50%
  • 10 year survival is 25-34%

More general terms

• oligodendroglial neoplasm

Additional terms

• oligodendrocyte

Patient information

oligodendroglioma patient information

Database

• OMIM: https://mirror.omim.org/entry/137800