Gliomas may be described as circumscribed or diffuse.
Circumscribed gliomas (e.g., pleomorphic xanthoastrocytoma, subependymal giant cell astrocytoma, pilocytic/pilomyxoid astrocytoma) have well-defined margins, and are generally benign. Maximal safe resection is the mainstay of treatment.
Diffuse astrocytomas (grade 2-4) represent a spectrum of the same highly infiltrative disease, with lower grades inevitably progressing to higher-grade lesions. They can be characterized on the basis of the isocitrate dehydrogenase (IDH) and histone 3 (H3) mutation status.
IDH-wildtype diffuse glioma (glioblastoma) is an aggressive primary brain tumor that presents in patients in the sixth or seventh decade of life.
IDH-mutant gliomas (including oligodendroglioma, IDH-mutant, 1p/19q codeleted, grades 2-3; and astrocytoma, IDH-mutant, grades 2-4) tend to occur in young or middle-age patients.
H3 mutant gliomas (including diffuse midline glioma, H3 K27M altered; and diffuse hemispheric glioma, H3 G34-mutant) are aggressive (grade 4) brain tumors occurring in children, adolescents, and young adults.
There are no known predisposing factors for gliomas except for rare familial syndromes and prior cranial radiation therapy.
Treatments vary depending on tumor grade and molecular characteristics. Initial treatment typically includes maximal safe surgical resection, with the aim of gross total resection if possible. Adjuvant radiation therapy and chemotherapy is indicated for most diffuse gliomas, with regimens varying based on molecular pathology, extent of resection, and other patient-specific factors.
Gliomas are primary tumors of the brain arising from glial progenitor cells. Normal glia (astrocytes and oligodendrocytes) provide structural and metabolic support to the brain and neurons, regulate ion concentrations in the extracellular space, modulate synaptic transmissions, and take up and release neurotransmitters. Gliomas may be circumscribed or diffuse.
History and exam
Other diagnostic factors
- altered mental status
- nausea and/or vomiting
- gait abnormality
- visual disturbances
- speech deficit
- sensory deficit
- motor weakness
- visual change
- cranial nerve palsy
- personality change/emotional lability
- white ancestry
- male sex
- neurofibromatosis type 1
- tuberous sclerosis complex
- Li-Fraumeni syndrome
- Turcot syndrome
- ionizing radiation
1st investigations to order
- MRI head
- ophthalmologic evaluation; visual field testing
- CT head
- brain MR spectroscopy
- brain perfusion MRI
- molecular analyses
Investigations to consider
- pituitary hormones tests
- diffusion tensor imaging (DTI)
- 2-hydroxyglutarate-targeted magnetic resonance spectroscopy
elevated intracranial pressure or vasogenic edema
circumscribed glioma: pilocytic/pilomyxoid astrocytoma
circumscribed glioma: subependymal giant cell astrocytoma (SEGA)
circumscribed glioma: pleomorphic xanthoastrocytoma
diffuse infiltrating glioma: grade 2
diffuse infiltrating glioma: grades 3 and 4
diffuse midline glioma, H3 K27M-altered
recurrent circumscribed glioma
progressive diffuse infiltrating glioma
- Brain metastasis
- Brain abscess
- Multiple sclerosis
- NCCN clinical practice guidelines in oncology: central nervous system cancers
- Therapy for diffuse astrocytic and oligodendroglial tumors in adults: ASCO-SNO guideline
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