Summary

• Benign non-glial epithelial CNS tumour, constituting 1% to 3% of intracranial tumours in adults, most often located near the optic chiasm.
• Bimodal age incidence with 50% in children aged between 5 and 14 years and a second peak in adults 50 to 70 years of age.
• Common acute presentations are sudden visual loss and symptoms of intracranial hypertension.
• Pituitary dysfunction is also common; children may present with growth failure and adults with diabetes insipidus and sexual dysfunction.
• Primary treatment consists of surgery. Following surgery, radiotherapy may be required.
• The majority of patients require adjunctive endocrine replacement therapy.