FACTS
- represent 10% gliomas
- age 35-40 years, M = F
- WHO Grade II or III
- diffusely infiltrating
- arise in white matter of hemispheres
A/P
- Standard of care: GTR + radiation + chemotherapy (RTOG 9802)
- Chemo: PCV (procarbazine, carmustine, vincristine)
- 1p/19q heterozygosity loss associated w/ better response
- Counsel:
- prognosis highly depends on grade (low grade 74% 5 year survival, high grade 41% 5 year)
- poor prognostic factors
- pseudo-palisading necrosis, TERT mutation, IDH WT
- favorable prognostic factors
- low-grade, combined loss of 1p19q, younger age, good KPS
Pathology
- sheets of similar cells with “perinuclear halos”
- Round nuclei with scant cytoplasm “chicken-wire vasculature”: occasional serpentine configuration, fried egg yolk appearing cells
- IHC: GFAP+/S100+
References
- Buckner, J. C. et al. Radiation plus Procarbazine, CCNU, and Vincristine in Low-Grade Glioma. New England Journal of Medicine 374, 1344–1355 (2016)
- Jhaveri J, et al. Is less more? Comparing chemotherapy alone with chemotherapy and radiation for high-risk grade 2 glioma: an analysis of the National Cancer Data Base