Central nervous system tumours (1)
Brain tumours are the most common
solid tumours, accounting for 25% of all childhood malignancies.
·
Involvement
of the multidisciplinary team is central to management of CNS tumours.
·
The
presenting features vary and may delay the diagnosis. For every childhood brain
tumour, there are 75000 children with migraine!
·
CT is
quick and available. It provides essential information for emergency management
of hydrocephalus.
·
MRI
gives better tumour definition. Combine with spinal imaging for staging of
disease.
This requires prompt treatment:
·
Referral
and transfer to a paediatric neurosurgical unit.
·
Control
tumour swelling with high dose steroids (usually dexamethasone).
·
CSF drainage: initial surgery may involve CSF
diversion only, biopsy, or complete
resection, depending on location and likely diagnosis.
·
Most
are pilocytic astrocytoma.
·
Cerebellum
and optic pathway are most common sites.
·
Outcome
depends on site. Posterior fossa lesions can be cured with surgery alone,
whereas optic pathway tumours are relatively inaccessible and morbidity is
high.
·
50% of
optic pathway low grade gliomas.
·
Visual
outcome better.
Radiotherapy contraindicated -
increased risk of second tumours.
·Predominantly occur in older
children and teenagers.
·Supratentorial sites predominate.
·Difficult to manage since complete
resection, essential for good outcome, is difficult to achieve.
·Rarely cured. Treatment usually
includes radiotherapy, with the addition of temozolamide or other agents in the
context of clinical trials
·Glioma in the region of the pons,
usually high-grade and inoperable.
·Radiotherapy is the mainstay of
treatment.
·Median survival <1yr.
·Most common malignant brain
tumours of childhood.
·Majority occur in the cerebellum
(medulloblastoma).
·Peak incidence is <5yrs.
·Tumour metastases (mainly via the
CSF) in 10–15%.
·70% of localized cases can be
cured, but expect significant long-term morbidity from radiotherapy.
Treatment includes excision and
craniospinal radiotherapy. Additional chemotherapy carries a survival
advantage, allowing reduction in drug dose and/or field of radiotherapy,
particularly in younger patients. Chemotherapy regimens include alkylating
agents (e.g., Lomustine (CCNU), cyclophosphamide), platinum drugs (cisplatin,
carboplatin), and vincristine. These are usually given after radiotherapy.
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