Brain Tumours Flashcards
What is the epidemiology of brain tumours in childhood
Leading cause of childhood cancer deaths
Most common solid organ tumour in childhood
Majority are primary
60% infratentorial
Most cerebellum or posterior fossa
What are the types of childhood CNS tumours
Astrocytoma (40%)
Medulloblastoma (20%)
Ependymoma (8%)
Brainstem glioma (6%)
Craniopharyngioma (4%)
Atypical teratoid/rhabdoid tumour
Describe astrocytomas
Can be benign or highly malignant (glioblastoma multiforme)
Cerebellar
Most common is Pilocytic astrocytoma
- Common in neurofibromatosis (NF1)
- BRAF mutation present in 70% of cases
What are the features of pilocytic astrocytoma on investigation
MRI: cerebellar, well-circumscribed, cystic, enhancing
Histopathology:
- Piloid (hairy) cell
- Rosenthal fibres and granular bodies
- Slow growing with low mitotic activity
Describe medulloblastomas
Embryonal tumour: originates from neuroepithelial precursors
Arises in the midline of posterior fossa
Cerebellar
May seed through the CNS via the CSF
Up to 20% have spinal metastases at diagnosis
Describe ependymomas
Mostly in the posterior fossa where it behaves like a medulloblastoma
Describe brainstem gliomas
Malignant tumours associated with a very poor prognosis
Describe craniopharyngiomas
Developmental tumour arising from the squamous remnant of Rathke pouch.
It is not truly malignant but is locally invasive
It grows slowly in the suprasellar region
Describe Atypical teratoid/rhabdoid tumour
Rare type of aggressive tumour that most commonly occurs in young children
What are the symptoms of brain tumours
Varies according to age, but usually:
Persistent or recurrent vomiting
Problems with balance, coordination or walking
Behavioural changes
Abnormal eye movements
Seizures (without fever)
Abnormal head position- wry neck, head tilt or persistent stiff neck
Infants: developmental delay/regression, irritability, lethargy, FTT, increase in head circumference/sutures/fontanelle
Children/adolescents: Persistent/recurrent headache (worse in morning.coughing), lethargy, blurred/double vision, deteriorating school performance, growth failure
What are the symptoms of spinal tumours
Back pain
peripheral weakness of arms/legs
Bladder/bowel dysfunction
What are the signs of a brain tumour in children
Raised ICP: disc oedema (papilloedema), obscuration of margins, elevation, venous congestion, haemorrhages
Cushing’s triad (late sign - likely to be coning by this time): Hypertension, bradycardia, deep breathing (pressure on the medulla)
Note: Papilloedema may be present but can be a late sign and difficult to detect
What is the difference in presentation between intracranial, supratentoral and subtentorial brain tumours
Intracranial HTN: headache, vomiting, changed mental state
Supratentorial: focal neurological deficits, seizures, personality change
Subtentorial: cerebellar ataxia, long tract signs, cranial nerve palsies
What investigations should be done for brain tumours in children
MRI
LP: ?CSF mets
What is the management for brain tumours in children
MDTI: paediatrician, neurologist, SN, OT, PT, SALT, psychology, radiologist, oncologist, CLIC Sargent (Cancer and leukaemia in Children social worker)
Surgery: maximal safe resection
- Craniotomy (debulking)
- Open biopsies (inoperable but approachable)
- Stereotactic (open biopsy not indicated)
Radiotherapy and chemotherapy
What are the complications of brain tumours
Children with brain tumours are at particular risk of:
- Neurological disability
- Growth, endocrine, neurophysiological and educational problems
due to potential site of the tumour, hazards of surgery and radiotherapy treatment given.
What is a neuroblastoma
Arises from neural crest tissue in the adrenal medulla and sympathetic nervous system
Spectrum of disease from benign (ganglioneuroma) to highly malignant (neuroblastoma)
Can spontaneously regress in young infants
What are the signs and symptoms of neuroblastomas
Pallor
Weight loss
Abdominal mass (or anywhere on the sympathetic chain from neck to pelvis) - Crosses the midline
Hepatomegaly
Bone pain
Limp
Rare symptoms: paraplegia, cervical lymphadenopathy, proptosis, periorbital bruising, skin nodules
Paravertebral tumour → spinal cord compression
>2yo: symptoms usually from mets (bone pain, BM suppression, malaise)
What investigations should be done for neuroblastoma
CT/MRI
Urine catecholamine metabolites (VMA and HVA): high
Biopsy
BM sampling
MIBG scan: labelling of neuroblastoma cells with radioactive iodine
What is the management for neuroblastoma
Some may resolve spontaneously
Localised primary: Cure with surgery
Metastatic disease: chemotherapy, high-dose therapy with Autologous stem cell rescue, surgery, radiotherapy (high risk of relapse)
What is the prognosis for neuroblastoma
Most children presenting > 1 year of age present with advance disease and have a POOR prognosis
Cure rates for children with metastatic disease is around 40%
Amplification of the MYCN oncogene predicts aggressive behaviour
