Central nervous system tumours (2)
·
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.
·
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.
·
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.
·
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.
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