Medulloepithelioma | |
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Histopathology of medulloepithelioma showing characteristic neural tube like strands. | |
Classification and external resources | |
ICD-O | M9501/3 |
MeSH | D018242 |
Medulloepithelioma is a rare, primitive, fast-growing brain tumour thought to stem from cells of the embryonic medullary cavity. Tumours originating in the ciliary body of the eye are referred to as embryonal medulloepitheliomas, or diktyomas.
A highly malignant undifferentiated primitive neuroepithelial tumour of children, medulloepithelioma may contain bone, cartilage, skeletal muscle, and tends to metastasize extracranially.
Medulloepithelioma most commonly affect children between 6 months and 5 years; rarely, this tumour may occur congenitally or beyond this age range.Incidence is equal in males and females.
Medulloepithelioma was originally classified as the most primitive neoplasm of the Central Nervous System (CNS) by Bailey and Cushing in 1926. Rorke et al. classified this tumour into two subtypes:
Medulloepithelioma have been reported to occur in the cerebral hemispheres, brainstem, cerebellum, and peripheral sites.
Due to rapid growth of the tumour, patients typically present with increased intracranial pressure, seizures, and focal neurologic signs.