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glial neoplasm (glioma)

Etiology: - prior exposure to radiation therapy only consistent risk factor [1] Epidemiology: - 50% of all primary brain neoplasms - most commonly occur in 4th-6th decade of life - grade 3 & 4 most commonly occur in patients 40-70 years Genetics: - down-regulation of BTBD10, CHN2 - overexpression of PHF19, MDM2 - ARHGAP29 is up-regulated in migrating glioma cells - other implicated genes: XRRA1, IIP45 Clinical manifestations: - seizures is most common presenting symptom - patients may present with focal neurologic deficit Special laboratory: - tissue diagnosis by brain biopsy or craniotomy & resection Radiology: - MRI with gadolinium contrast is imaging modality of choice a) low-grade gliomas appear lesions with 1] indistinct margins 2] minimal contrast enhancement 3] little or no edema b) higher grade gliomas are more likely to show contrast enhancement & surrounding edema c) oligodendrogliomas are generally calcified (90%) * image [6] Staging: - grade 1: low grade - grade 2: a) low grade, grows slowly b) inevitably transforms into higher grade if left untreated - grade 3: high grade - grade 4: glioblastoma multiforme Management: - surgical resection is curative in patients with grade 1 gliomas - grade 2 & 3 gliomas: surgical resection followed by radiation therapy &/or chemotherapy [2,3] - see astrocytoma - see oligodenroglioma - see glioblastoma multiforme - prognosis: a) median survival is 9 years for grade 2 gliomas b) median survival is 3 years for grade 3 gliomas c) median survival is 1 year for grade 4 gliomas (glioblastoma multiforme) d) predictors of poor prognosis 1] advanced age, 2] poor performance status 3] larger tumor size 4] tumors crossing the midline 5] astrocytic histopathology 6] higher WHO grade

Specific

astrocytoma (astrocytic neoplasm) chordoid glioma of the third ventricle ependymal neoplasm ganglioglioma; dysembryonic neuroepithelial neoplasm gliomatosis cerebri malignant glioma mixed glioma oligodendroglial neoplasm

General

neuroepithelial neoplasm (includes glial, pineal, choroid plexus, ganglion cell neoplasms and others)

References

  1. Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 2009, 2012, 2015, 2018, 2021.
  2. Kaloshi G et al. Supratentorial low-grade gliomas in older patients. Neurology 2009 Dec 15; 73:2093. PMID: 19907009
  3. Smith JS, Chang EF, Lamborn KR et al Role of extent of resection in the long-term outcome of low-grade hemispheric gliomas. J Clin Oncol. 2008 Mar 10;26(8):1338-45 PMID: 18323558
  4. Surma-aho O, Niemela M, Vilkki J et al Adverse long-term effects of brain radiotherapy in adult low-grade glioma patients. Neurology. 2001 May 22;56(10):1285-90. PMID: 11376174
  5. Weller M, Stupp R, Hegi ME et al Personalized care in neuro-oncology coming of age: why we need MGMT and 1p/19q testing for malignant glioma patients in clinical practice. Neuro Oncol. 2012 Sep;14 Suppl 4:iv100-8 PMID: 23095825
  6. Khanna O, Ghobrial GM, Farrell CJ 10 Brain Lesions to Recognize (MRI images) Medscape. October 25, 2021 https://reference.medscape.com/slideshow/brain-lesions-6013313