brain tumours

brain tumours

in kids (vs adults)
1. almost always 1ry
2. 60% are infratentorial
commonest & solid tumour in kids
leading cause of childhood Ca deaths in UK
types
1. astrocytoma (~40% of cases)
  1. vary from benign to highly malignant (glioblastoma multiforme)
  2. Mx- surgery, radiotherapy +/- chemo
    1. but prognosis poor (<30% survival) @ cerebral site
  3. in cerebellum, slow growing & cystic
    1. good prognosis post surgery
2. medulloblastoma (~20%)
  1. arises in midline of post fossa
  2. may seed through CNS via CSF
  3. up to 20% have spinal mets @ diag
  4. Mx-surgery, chemo, radiotherapy
  5. prognosis- survival rates improving- 5 year survival about 50%
3. ependymoma (~8%)
  1. mostly in post fossa
    1. behaves like medulloblastoma
4. brainstem glioma (6%)
  1. not for biopsy- too risky
  2. very poor prognosis (<10% survival)
5. craniopharyngioma (4%)
  1. developmental tumour arising from squamous remnant of Rathke pouch
  2. not truly malignant but locally invasive & grows slows in suprasellar region
  3. Mx- surgical excision +/- radiotherapy
  4. prog- good survival but risk of long term visual imp & lifelong pituitary insuff
clinical features
1. location of tumours
  1. supratentorial
    1. cortex
      1. diag- astrocytoma
        commonly in cerebral hemispheres, thalamus & hypothalamus
      2. seizures
      3. hemiplegia
      4. focal neurological signs
    2. typical hx
      1. 14 year old, aggressive behaviour @ school, headaches, seizure
  2. midline
    1. diag- craniopharyngioma
    2. visual field loss- bitemporal hemianopia
    3. pituitary failure
      1. growth failure
      2. diabetes insipidus
      3. weight gain
    4. typical hx
      1. 10 year old, c/o headaches, vomiting, poor growth, hard to see board @ school
  3. infratentorial
    1. cerebellar (& 4th ventricle)
      1. diag
        medulloblastoma
        ependymoma
        astrocytoma
      2. abnormal eye movements
      3. truncal ataxia
      4. coordination difficulties
      5. typical hx
        3 year old vomiting in morning, unsteady on feet, new onset convergent squint
    2. brainstem
      1. diag
        brainstem glioma
      2. cranial nerve defects
      3. pyramidal tract signs
      4. cerebellar signs- ataxia
      5. often no raised ICP
      6. typical hx
        4 year old, refuses to walk, unable to climb stairs, squint, facial asymmetry & drooling
  4. spinal cord
    1. diag
      1. astrocytoma
      2. ependymoma
    2. primary or mets (depends on level of lesion)
      1. back pain
      2. peripheral weakness of arms or legs
      3. bladder/bowel dysfunction
2. raised intracranial Pa
  1. kids & teens
    1. headaches-worse in morning
    2. vomiting- esp on waking in morning
    3. behaviour/personality change
    4. visual disturbance
    5. papilloedema
  2. infants
    1. vomiting
    2. separation of sutures/tense fontanelle
    3. head tilt/posturing
    4. developmental delay/regression
    5. increased head circumference
Ix
1. MRI
  1. best for characterising brain tumours
2. magnetic resonance spectroscopy
  1. to examine biological activity of tumour
3. lumbar puncture
  1. not to be done w/o neurosurg advice if any ?raised ICP
Mx
1. surgery
  1. usually 1st Rx
  2. aims
    1. treating hydrocephalus
    2. providing tissue diagnosis
    3. attempting max resection
2. anatomical location may mean tumour can't be biopsied
  1. brainstem tumours
3. even histologically 'benign' tumours can be challenging to survival
4. use of radiotherapy +/- chemo varies w/ tumour type & pt age
late effects
1. risks of
  1. neurodisability
  2. growth, endocrine, neuropsychological, educational probs

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	Creado por v.djabatey hace más de 10 años