medulloblastoma

Last reviewed 01/2018

A medulloblastoma is a tumour arising from embryonic tissue in the posterior part of the cerebellar vermis. 70% of patients present before 8 years of age and it is twice as common in boys than girls. It is the most common intracranial malignant tumour of childhood and constitutes about 20% of all intracranial tumours.

It is one of the few intracranial tumours that metastasise with spinal involvement in about 40% of newly diagnoses cases. Exfoliated malignant cells are often discernible in the CSF.

• the last 30 years have seen significant improvements in overall survival, at around 80% for patients with average risk (children >3 years of age, less than 1.5 cm2 of residual disease in the primary site, and absence of metastatic disease) and 60%-70% for high-risk patients (those with bulky residual disease, brain stem invasion, and neuraxis metastasis) with the use of standard chemotherapy and radiotherapy (craniospinal irradiation and focal boost)

Reference:

• Pui CH, Gajjar AJ, Kane JR, Qaddoumi IA, Pappo AS. Challenging issues in pediatric oncology. Nat Rev Clin Oncol. 2011;8:540-549
• Siegel R, Naishadham D, Jemal A. Cancer statistics, 2012. CA Cancer J Clin. 2012;62:10-29.