Neurosurgery notes/Tumours/Tumour general/Paediatric Cranial tumours/Congenital brain tumours <60days

Congenital brain tumours <60days

View Details
Status
Done
logo
Parent item
Paediatric cranial tumours

Numbers

  • Extremely rare, 0.5-1.5%
  • Improved fetal USG and fetal/neonatal MRI
  • Distinct histological AND clinical behaviour than tumours of infancy 1-2yo
  • The most common type is teratoma (26.6% to 48%)
    • Frequently occur before 32wks of gestation
    • Resembles normal embryonic development
    • Aberrant and residual cells during the formation of CNS
  • Others:
    • Astrocytoma (7.4% to 28.8%)
    • Choroid plexus papilloma (3.7% to 13.2%)
    • Embryonal tumor (3% to 13%)
notion image
 

Types of tumours

Teratoma

  • >50% cases
  • All 3 germ cell layers
  • Mature or immature
  • Midline, pineal region
notion image

Choroid plexus tumour

  • 70% diagnosed <2yo
  • 80% in lateral ventricle
  • Pathology
    • Choroid plexus papilloma (WHO 1)
    • Atypical (WHO2)
    • Choroid plexus carcinoma (WHO 3)
  • Aicardi syndrome
    • X-linked dominant
    • MRI – corpus callosum agenesis, infantile spasms
  • Li-Fraumeni syndrome
notion image

Astrocytoma

Brainstem gliomas
  • Brainstem gliomas
  • Up to 10-20% of all CNS paediatric tumours
  • DIPG most common, present at 5-9 yo
  • Average OS only 9-12 months
  • NF1 better prognosis
ㅤ
DIPG (75-80%)
Focal
Age
5-10yo
Older age
MRI
Homogeneous, diffuse tumour at pons contrast+ highly variable
Medulla/cervicomedullary tectal glioma dorsal exophytic growth
Pathology
High grade glioma (WHO 3 or 4) molecular: TP53, H3K27M-mutant
Pilocytic astrocytoma Ganglioglioma
Treatment
MRI diagnosis, NO BIOPSY shunt
Subtotal removal of dorsal exophytic lesion shunt
Adjuvant Treatment
Direct RT + chemo Steroid RT 44-55Gy/30Fr (in 6 weeks) - 80% improve but decline rapidly afterwards +/- Temodal
Reoperation if recur (remain histologically benign) or RT
Survival
Die in 1 yr
5yr OS >90
High grade gliomas
  • 6.5% of all paediatric intracranial tumours
  • Risk factors:
    • Genetic syndromes: NF-1, Turcot syndrome, Li-Fraumeni
    • Previous irradiation for haematological malignancies
  • Differ in molecular characteristics and prognostic implications from adults
    • High incidence 40.5% of TP53 mutation
    • Less EGFR, PTEN mutation
    • H3F3A histone gene mutation
  • Very different genetic makeup of these tumour when compared to adults
  • Treatment
      • Aggressive maximal safe resection
      • ChemoRT
      • Future target therapies
      notion image

Pituitary stalk and pineal region

  • Failure to thrive
  • Growth failure
  • Diabetes insipidus
    • Pre-operative DI unusual in craniopharyngioma
    • But common in germ cell tumours involving anterior third ventricle, histiocytosis
  • Delayed puberty
  • Diencephalic syndrome
  • Rarely hypersecretory endocrine function
  • Visual problems
- Supranuclear upgaze palsy - Large pupils with light-near dissociation - Lid retraction (Collier sign)
- Supranuclear upgaze palsy
- Large pupils with light-near dissociation
- Lid retraction (Collier sign)
- Normal downgaze - Convergence weakness - Convergence-retraction nystagmus
- Normal downgaze
- Convergence weakness
- Convergence-retraction nystagmus

Treatment

  • Need for Caesarean section due to large head and risk of dystocia
  • Surgical resection
  • Chemotherapy
  • Poor survival
    • Less aggressive tumour resection due to blood loss
    • High grade pathology
    • Avoidance of radiotherapy
      • Severe intellectual and psychomotor retardation in immature brain