Discussion Definitions: Rapidly enlarging malignant astrocytic tumor characterized by necrosis and neova scularity.

Most common of all primary intracranial neoplasms. General Features Best diagnostic clue: Thick, irregular-enhancing rind of neoplastic tissue surro unding necrotic core. Location: Supratentorial white matter most common. Frontal, temporal, parietal lobes, occipital lobes relatively spared. Cerebral hemispheres> brainstem > cerebellum. Brainstem, cerebellum more common in children. Size: Variable Morphology: poorly-marginated, diffusely-infiltrating necrotic hemispheric mass. Tumor typically crosses white matter tracts to involve contralateral hemisphere. Corpus callosum ("butterfly glioma") Anterior and posterior commissures. May invade meninges (rarely). Rarely may be multifocal, multicentric CT Findings NECT 1. Irregular isodense or hypodense mass with central hypodensity representing ne crosis. 2. Marked mass effect and surrounding edema/tumor infiltration. 3. Hemorrhage not uncommon 4. Ca++ rare (related to low grade tumor degeneration) 5. CECT: 95% have strong, heterogeneous, irregular rim-enhancement MR Findings -TlWI: 1. Irregular isointense, hypointense white matter mass 2. Necrosis, cysts and thick irregular margin common 3. May have subacute hemorrhage -T2WI 1. Heterogeneous, hyperintense mass with adjacent tumor infiltration/vasogenic e dema. 2. Viable tumor extends far beyond signal changes! 3. Necrosis, cysts, hemorrhage, fluid/debris levels, flow voids (neovascularity) may be seen. -FLAIR: 1. Heterogeneous, hyperintense mass with adjacent tumor infiltration/vasogenic e dema. - T2* GRE: 1. Susceptibility artifact on T2* common related to blood products. - DWI: 1. Lower measured ADC than low grade gliomas. 2. No diffusion restriction typical. - T1 C+: 1. Thick, irregular rind of enhancement surrounding central necrosis typical. 2. Enhancement may be solid, ring, nodular or patchy - MRS: 1. -Decreased NAA, myo-inositol 2. Elevated choline, lactate/lipid peak (1.33 ppm) 3. Dynamic contrast-enhanced T2* weighted imaging 4. Elevated maximum rCBV compared to low grade 5. Increased permeability compared to low grade tumors, may help assess tumor gr ade.

6. Diffusion tensor imaging (DTI) of white matter tracts may help surgical plann ing in future Angiographic Findings - DSA: 1. Hypervascular mass, prominent tumor blush. 2. A-V shunting and early draining veins common. 3. Rarely may mimic an AVM. 4. Nuclear Medicine Findings - PET: 1. Malignant tumors have high glucose metabolism and avidly accumulate FDG, upta ke similar to or exceeding gray matter. 2. Tumor/WM > 1.5 and tumor/GM > 0.6 suggests high-grade tumors. 3. Imaging Recommendations -Best imaging tool: 1. MR is most sensitive 2. Newer techniques are being developed to improve diagnosis and biopsy accuracy : MRS, T2* imaging, perfusion, hypoxia imaging, PET, DTI. 3. Protocol advice: Contrast-enhanced MRi MRS, DWI may be helpful. DDX: 1. Abscess 2. Metastasis 3. Primary CNS lymphoma 4. Anaplastic astrocytoma (AA) 5. "Tumefactive" demyelination 6. Subacute ischemia 7. Status epilepticus 8. Arteriovenous malformation (AVM) General Features: -General path comments: - Two types, primary (de novo) and secondary: degeneration from lower grade astr ocytoma. - Genetically distinct, same appearance - Giant cell glioblastoma, a histologic variant of GBM, (5%), slightly improved prognosis. - Genetics 1. Primary GBM (de novo): 1. 2. 3. 4. 5. Older patients, biologically more aggressive Develops de novo (without pre-existing lower grade tumor) Amplification, overexpression of EGFR, MDM2 PTEN mutation Chromosome lOp loss of heterozygosity (LOH)

2. Secondary GBM (degeneration from lower grade) 1. Younger patients, less aggressive than primary GBM. 2. Develops from lower grade astrocytoma. 3. TP53 mutations. 4. PDGFR amplification, overexpression. 5. Chromosomes 109, 17p LOH. 6. Increased telomerase activity and hTERT expression. 7. Occurs sporadically or as part of heritable tumor syndrome: NFl, Li-Fraumeni syndrome (p53 mutation) ,Turcot syndrome, Ollier disease, Maffucci syndrome. -Image Interpretation Pearls: Viable tumor extends far beyond signal abnormaliti es! Corpus callosum involvement may be seen in GBM, lymphoma, and rarely metasta

ses, demyelination