Paediatrics: Central nervous system tumours (2)

Central nervous system tumours (2)

Ependymoma (10% of CNS tumours)

·  Periventricular sites.

·  Usually present with obstructive hydrocephalus.

·  10% metastasize to the spine.

·  Treated by surgical excision and involved field radiotherapy.

·  Chemotherapy used in younger patients to delay radiotherapy.

·  >70% survival if complete excision.

CNS germ cell tumours (5% of CNS tumours)

·  Rare and more commonly seen in teenage males.

·  Midline (suprasellar or pineal): 60% of malignant cases are germinoma, 40% non-germinomatous (secreting) malignant GCTs (GCTs; e.g. embryonal carcinoma, yolk sac tumour, mixed malignant tumours). Mature teratomas seen more in younger patients.

·  Secreting tumours: characterized by raised markers (AFP or hCG) in either serum or CSF. (Biopsy in marker –ve cases).

·  Primary surgery for teratoma: chemotherapy and radiotherapy for other tumour types.

·  Cure in 70% secreting tumours and >90% for germinoma/teratoma.

Craniopharyingioma (10% of CNS tumours)

·  Slow-growing midline epithelial tumours in the suprasellar area from ‘Rathke’s pouch’.

·  Treatment: complete resection in 80%, partial resection with focal radiotherapy in the remainder. Complications include damage to the hypothalamic–pituitary structures, vision, and behaviour.

Retinoblastoma (3% of all tumours)

·  Sporadic or familial (40%) forms that are unilateral or bilateral (30%) on presentation.

·  Peak incidence: unilateral disease, 2–3yrs; bilateral disease, 0–12mths.

·  Presentation: absent or abnormal light reflex (leucocoria), squint, or visual deterioration.

·  Treatment: surgery, chemotherapy, and focal therapy.

·  90% 5-year survival: inherited form at risk of second p malignancy, with OS being the most common.