CNS Tumours

Question 1

Define

Answer 1

In children, the majority of these are primary, 60% are infratentorial

· Most common solid organ tumour in childhood à leading cause of childhood cancer deaths

Astrocytoma (~40%)

• Varies from benign to highly malignant (glioblastoma multiforme)
• Cerebellar

Pilocytic astrocytoma
* Most common child brain tumour (20% CNS tumours <14yo)
* Common in neurofibromatosis I (NF1)
* MRI: cerebellar; well circumscribed, cystic, enhancing

Histopathology:
* Piloid (hairy) cell
* Rosenthal fibres and granular bodies
* Slow growing with low mitotic activity

BRAF mutation present in 70% of cases

Question 2

Symptoms

Answer 2

Presentation varies according to age and their ability to report symptoms:

All ages
* 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

Child/ Adolescent
* Persistent or recurrent headache
* Worse in morning/ coughing
* Blurred or double vision
* Lethargy
* Deteriorating school performance
* Delayed or arrested puberty, slow growth

Infants
* Developmental delay/ regression
* Irritable
* Failure to thrive
* Progressive increase in head circumference, separation of sutures, bulging fontanelle
* Lethargy

Signs and symptoms often relate to raised ICP
* disc oedema, obscuration of margins, elevation, venous congestion, haemorrhages

Focal neurological signs may be detected depending on the size of the tumour
1. Intracranial HTN: headache, vomiting, changed mental state
2. Supratentorial: focal neurological deficits, seizures, personality change
3. Subtentorial: cerebellar ataxia, long tract signs, cranial nerve palsies

Papilloedema may be present but can be a late sign and difficult to detect

Spinal tumours (primary or metastatic) can present with back pain, peripheral weakness of arms or legs, or bladder/ bowel dysfunction, depending on the level of the lesion

Question 3

Investigations

Answer 3

MRI - (Magnetic resonance spectroscopy can be used to examine the biological activity of a tumour)

Lumbar puncture - (reveals metastases within the CSF)

Question 4

Management

Answer 4

MDT: paediatrician, neurologist, SN, OT, PT, SALT, psychology, radiologist, oncologist, CLIC Sargent
* CLIC Sargent = Cancer and Leukaemia in Children social worker

SURGERY
* maximal safe resection to obtain and extensive excision with minimal damage to the patient
* Resectability is dependent on the location, site and number of lesions
* Craniotomy  debulking (subtotal and complete resections)
* Open biopsies  inoperable but approachable tumours
* Stereotactic biopsy  open biopsy not indicated

Radiotherapy and chemotherapy
* The use of this varies with tumour type and the age of the patient

Question 5

Complications/ Prognosis

Answer 5

Children with brain tumours are at particular risk of neurological disability and of growth, endocrine, neurophysiological and educational problems due to potential site of the tumour, hazards of surgery and radiotherapy treatment given.

Survivors may have complex combinations of these problems