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Updated: Apr 2 2022

Primary Brain Tumors

  • Overview
    • Presentation
      • Most common complaints are due to mass effects
        • e.g., seizures, dementia, focal lesions, headache
      • majority of adult primary tumors are supratentorial
      • majority of childhood primary tumors are infratentorial
    • Primary tumor characteristics
      • rarely undergo metastasis
      • usually poorly circumscribed
      • primary brain tumor frequency
        • meningioma > pituitary tumor > glioblastoma > nerve sheath tumor
      • risk factors
        • NF, cigarette smoking, Turcot's syndrome (colonic polyps and brain tumor)
    • Secondary tumor characteristics
      • 50% of adult brain tumors are due to metastases
      • well circumscribed
      • usually present at gray-white junction
      • frequency of primary tumor causes
        • lung > breast > melanoma (skin) > kidney > GI
  • Adult peak-incidence tumors
    • Glioblastoma multiforme
      • type: grade IV astrocytoma
      • prognosis: grave (< 1 year life expectancy)
      • location: cerebral hemispheres
      • stain: GFAP
      • histologic appearance: "psuedopalisading" pleomorphic tumor cells
        • border central areas of necrosis and hemorrhage
      • gross appearance: can cross corpus callosum (butterfly glioma)
      • note: may arise de novo or progress from a lower grade astrocytoma
        • methylation of MGMT gene is a positive prognostic factor for survival
    • Meningioma
      • prognosis: resectable
      • location: convexities of hemispheres , parasagittal region, olfactory groove
        • arises from arachnoid cells covering brain
      • histological appearance: spindle cells concentrically arrange in a whorled pattern
        • psammoma bodies (laminated calcifications)
      • note: more common in women (due to estrogen receptors on tumor cells)
        • associated with neurofibromatosis
    • Schwannoma
      • prognosis: good, resectable
      • location: often localized to CN VIII → acoustic schwannoma
        • usually found at cerebrellopontine angle
        • can present with tinnitus, hearing loss
      • staining: S-100 positive
      • histologic appearance: biphasic with hypercellular (Antoni A) and hypocellular (Antoni B) areas
      • note: bilateral schwannoma found in neurofibromatosis type 2
    • Oligodendroglioma
      • prognosis: good, slow growing
      • location: most often in frontal lobes
      • histologic appearance: chicken-wire capillary pattern with "fried egg" cells
        • round nuclei with clear cytoplasm
      • gross appearance: often calcified in frontal lobe
  • Childhood peak-incidence tumors
    • Pilocytic (low-grade) astrocytoma
      • type: grade I astrocytoma
      • prognosis: benign
      • location: most often infratentorial
        • may be supratentorial (most commonly, craniopharyngioma)
      • stain: GFAP
      • histologic appearance: Rosenthal fibers
        • eosinophilic corkscrew fibers
      • gross appearance: cystic + solid, usually well-circumscribed
    • Medulloblastoma
      • most common malignant intracranial tumor of childhood
      • prognosis: poor, highly malignant
        • form of primitive neuroectodermal tumor (PNET)
      • location: cerebellum
        • can compress 4th ventricle → hydrocephalus
      • histologic appearance: rosettes with small blue cells
      • gross appearance: solid
      • note: radiosensitive
    • Ependymoma
      • prognosis: poor
      • location: most commonly in 4th ventricle
        • can result in hydrocephalus
        • can also arise from cauda equina in adults
        • arises from ependymal cells
      • histologic appearance: characteristic perivascular pseudorosettes
    • Hemangioblastoma (occurs typically in adults if no genetic predisposition as below)
      • location: cerebellar
      • histologic appearance: foamy cells and high vascularity
      • note: associated with von Hippel-Lindau syndrome when found with retinal angiomas
        • can produce EPO → secondary polycythemia
    • Craniopharyngioma
      • prognosis: benign
      • location: near sella turcica
        • can present with optic chiasm compression → bitemporal hemianopia
          • confused with pituitary adenoma
        • derived from remnants of Rathke's pouch
      • gross appearance: calcification is common, tooth enamel-like
    • Germinoma
      • prognosis: good
      • location: pineal gland and suprasellar region
        • derived from nests of embryonic cells arrested during their migration in fetal development
        • Pineal region: Parinaud syndrome (30-40% of cases) compression of tectum causes: paralysis of upward gaze, loss of light perception and accomodation, nystagmus, failure of convergence
        • Suprasellar region: anterior hypopituitarism, precocious puberty, diabetes inspidius, visual disturbances
      • stain: AFP and b-HCG
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