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Medulloblastoma

Medulloblastoma is a highly malignant small round blue cell tumor of the posterior fossa

Citation:
Gottardo NG, Howell CI. Medulloblastoma. In: Gajjar A, Reaman GH, Racadio JM, Smith FO, editors. Brain Tumors in Children. Cham: Springer International Publishing; 2018. p. 131-75.

Abstract:
Medulloblastoma is a highly malignant small round blue cell tumor of the posterior fossa. It accounts for approximately 20% of all brain tumors in children, is the most common malignant brain tumor of childhood and, with greater advances against other common cancers in children, such as acute leukemias, is currently responsible for about 10% of all childhood cancer deaths. Around 30% of patients present with met­astatic disease, which is essentially restricted to the central nervous system, with metasta­sis outside the brain and spinal cord reported in less than 1 % of cases at diagnosis in large series. Since the 1970s the applica­tion of multimodal therapy including maximal resection followed by craniospinal irradiation and chemotherapy have transformed a dis­ease that was invariably fatal when treated with surgery alone to one which can be cured for approximately 70% of all patients. The stratifica­tion of patients based on age, amount of residual tumor, and the presence of metastatic disease has traditionally classified patients into three distinct clinical groups: average-risk medulloblastoma, high-risk medulloblastorna, and infant medullo­blastoma. High-risk disease occurs in children >3 years old with < 1.5 cm2 of residual tumor and/or metastatic disease, while high-risk disease occurs in children >3 years old with >1.5 cm2 of residual tumor or metastatic disease.