Medulloepitheliomas are rare, highly malignant, primitive embryonic tumor derived from the primitive medullary plate and neural tube. It is essentially a tumor of childhood with the age of onset between 6 months and 5 years.
Medulloepitheliomas are classified as embryonal tumors in the WHO classification of CNS tumors 3.
On non-contrast CT scan, the lesion is either isodense or hypodense with variable heterogeneity and calcification. The tumor enhances with contrast.
Treatment and prognosis
Medullary epithelioma carries dismal prognosis with a median survival of 5 months. On the other hand, medulloepitheliomas occurring in the eye or the orbit generally benefit from a gross-total resection and may present a good prognosis.
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- 2. Russel DS, Rubinstein LJ. Pathology of tumors the nervous system, 5th edn. Baltimore: Williams & Wilkins 1989; pp. 247-51
- 3. Louis DN, Perry A, Reifenberger G et-al. The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol. 2016;131 (6): 803-20. doi:10.1007/s00401-016-1545-1 - Pubmed citation