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A primitive, fast-growing brain tumour thought to stem from cells of the embryonic medullary cavity of bone shafts. It is a highly malignant embryonal tumour of the central nervous system. It can grow in the cerebral hemispheres, brainstem, cerebellum, and peripheral sites. It most commonly affects children between 6 months and 5 years. Incidence is equal in males and females. (Types) • cerebral medulloepithelioma: A tumour that occurs in the brain or spinal cord (usually in the supratentorial region) of infants and young children (peak incidence between 6 months to 5 years of age). • diktyoma (ciliary body medulloepithelioma, medulloepithelioma of the optic disc and optic nerve): A tumour originating in the ciliary body or rarely, optic disc and optic nerve. (Symptoms: cerebral medulloepithelioma) • headache, nausea, and vomiting • increased intracranial pressure • seizures • focal neurologic signs: Perceptual or behavioral impairments which are caused by lesions in a particular area of the central nervous system. (Symptoms: diktyoma) • blurring of vision or vision loss (most common) • pain (most common) • leukocoria (most common): Presence of a mass in the iris or ciliary body. • lens subluxation • glaucoma • cataract • exophthalmos • buphthalmos • strabismus • ptosis (Diagnosis) • medical imaging: CT, MRI, ultrasound B-scan, fluorescein angiography • medical imaging and blood test of the abdomen: Detect metastases. • needle aspiration biopsy: Neural tube-like structures (mostly vimentin positive) or Flexner-Wintersteiner rosettes may be observed. (Treatment) • cerebral medulloepithelioma: Maximal safe resection of the tumour followed by radiation therapy with or without chemotherapy. (Radiation therapy alone to prolong survival.) • diktyoma: Enucleation of the eye. • metastatic medulloepithelioma: Aggressive chemotherapy with autologous bone marrow transplant. (Prognosis) • cerebral medulloepithelioma: Very poor prognosis with a median survival of 5 months. • diktyoma: Good prognosis after resection of intraocular tumours.