Medulloblastoma
Definition: Medulloblastoma is the most common embryonal neuroepithelial tumor arising in the cerebellum or posterior brain stem and most common malignant tumor of childhood consisting of densely packed small and round undifferentiated cells with high mitotic count.
Epidemiology: This is the most common CNS embryonal tumor of childhood and the second most common pediatric brain tumors after pilocytic astrocytoma; it accounts for up to 25% of all intracranial neoplasms in children. The median age at diagnosis of medulloblastoma is 9 years, with peaks incident at 3 and 7 years of age; it is more common in boys, with female-to-male ratio of 1:1.7. Among patients aged >3 years, the female-to-male ratio is 1:2. The various molecular groups and histopathological variants of medulloblastoma have different age distributions.
Molecular and genetic profile: Medulloblastomas are divided into four distinct molecular subtypes based on molecular profiles: WNT-activated, sonic hedgehog (SHH)-activated, non-WNT/non-SHH-activated group 3, and non-WNT/non-SHH-activated group 4.
Clinical features and standard therapy
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