![]() Standard treatment includes surgery, chemotherapy, and, depending on the age of the patient, radiation therapy (Crawford et al., 2007). Medulloblastoma is thought to arise from neural stem cell precursors in the granular cell layer of the cerebellum. Medulloblastoma also occurs in up to 40% of patients with Turcot syndrome (see 276300). In 1 to 2% of patients, medulloblastoma is associated with Gorlin syndrome (109400), a nevoid basal carcinoma syndrome. Medulloblastoma accounts for less than 1% of central nervous system (CNS) tumors in adults, with highest incidence in adults 20 to 34 years of age. Approximately 10 to 15% of medulloblastomas are diagnosed in infancy. ![]() ![]() Medulloblastoma occurs bimodally, with peak incidences between 3 and 4 years and 8 and 9 years of age. It accounts for 16% of all pediatric brain tumors, and 40% of all cerebellar tumors in childhood are medulloblastoma. Medulloblastoma is the most common brain tumor in children. Each subgroup is characterized by a unique set of genetics and gene expression as well as demographic and clinical features. At the molecular level, medulloblastomas have been categorized into the following subgroups: wingless (WNT), sonic hedgehog (SHH), group 3, and group 4. The latter 2 groups have been shown to have a significantly superior prognosis as compared to the large cell and anaplastic groups in young children. Pretreatment prognosis of medulloblastoma has been refined by histopathologic subclassification into the following variants: large-cell medulloblastoma, anaplastic medulloblastoma, desmoplastic/nodular medulloblastoma, and medulloblastoma with extensive nodularity (MBEN). Millard and De Braganca (2016) reviewed the histopathologic variants and molecular subgroups of medulloblastoma. ![]() ![]()
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