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Nervous System Disorders

= malignant tumors of glial cells growing along white matter tracts with tendency to increase in grade with time; may be multifocal

Incidence: 30–40% of all primary intracranial tumors; 50% of solitary supratentorial masses

CELL OF ORIGIN

1. AstrocyteAstrocytoma
2. OligodendrocyteOligodendroglioma
3. EpendymaEpendymoma
4. MedulloblastMedulloblastoma; (PNET = primitive neuroectodermal tumor)
5. Choroid plexusChoroid plexus papilloma

FREQUENCY OF INTRACRANIAL GLIOMAS

Age peak: middle adult life

Location: cerebral hemispheres; spinal cord; brainstem + cerebellum (in children)

Brainstem Glioma!!navigator!!

Incidence: 1%; 12–15% of all pediatric brain tumors; 20–30% of infratentorial brain tumors in children

Histo: usually anaplastic astrocytoma / glioblastoma multiforme with infiltration along fiber tracts

Age: in children + young adults; peak age 3–13 years; M÷F = 1÷1

  • become clinically apparent early before ventricular obstruction occurs
  • ipsilateral progressive multiple cranial nerve palsies
  • cerebellar dysfunction: ataxia, nystagmus
  • contralateral hemiparesis, eventually respiratory insufficiency

Location: pons >midbrain >medulla; often unilateral at medullopontine junction

  • Medullary + mesencephalic gliomas are more benign than pontine gliomas!

Growth pattern:

  1. diffuse infiltration of brainstem with symmetric expansion + rostrocaudal spread into medulla / thalamus + spread to cerebellum
  2. focally exophytic growth into adjacent cisterns (cerebellopontine, prepontine, cisterna magna)
  • asymmetrically expanded brainstem
  • flattening + posterior displacement of 4th ventricle + aqueduct of Sylvius
  • compression of prepontine + interpeduncular cistern (in upward transtentorial herniation)
  • paradoxical widening of CP angle cistern with tumor extension into CP angle
  • paradoxical anterior displacement of 4th ventricle with tumor extension into cisterna magna

CT:

  • isodense / hypodense mass with indistinct margins
  • hyperdense foci (= hemorrhage) uncommon
  • absent / vague (minimal / patchy) contrast enhancement (50%)
  • ring enhancement in necrotic / cystic tumors (= most aggressive tumors)
  • prominent enhancement in exophytic lesion
  • hydrocephalus uncommon (because of early symptomatology)

MR: (best evaluation in subtle cases)

  • hypointense on T1WI + hyperintense on T2WI
  • often only subtle enhancement
  • ± engulfment of basilar artery

Angio:

  • anterior displacement of basilar artery + anterior pontomesencephalic vein
  • posterior displacement of precentral cerebellar vein
  • posterior displacement of posterior medullary + supratonsillar segments of PICA
  • lateral displacement of lateral medullary segment of PICA

Prognosis: 10–30% 5-year survival rate

Rx: radiation therapy

DDx: focal encephalitis, resolving hematoma, vascular malformation, tuberculoma, infarct, multiple sclerosis, metastasis, lymphoma

Hypothalamic-Chiasmatic Glioma!!navigator!!

Origin: often undeterminable: hypothalamic gliomas invade chiasm, chiasmatic gliomas invade hypothalamus

Incidence: 10–15% of supratentorial tumors in children

Age: 2–4 years; M÷F = 1÷1

Associated with: von Recklinghausen disease (20–50%)

  • diminished visual acuity (50%) optic atrophy
  • diencephalic syndrome (in up to 20%): marked emaciation, pallor, unusual alertness, hyperactivity, euphoria
  • short stature (in 20%) reduction in growth hormone
  • obese child, sexual precocity, diabetes insipidus
  • suprasellar hypodense lobulated mass with dense inhomogeneous enhancement
  • heterogeneous lesion cyst formation, necrosis, calcifications
  • hypointense on T1WI + hyperintense on T2WI + FLAIR
  • obstructive hydrocephalus

DDx: hypothalamic hamartoma, ganglioglioma, choristoma


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