Medulloblastoma

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:

1. Orr BA. Pathology, diagnostics, and classification of medulloblastoma. Brain Pathol. 2020 May;30(3):664-678
2. Louis DN, Perry A, Wesseling P, et al. The 2021 WHO classification of tumors of the central nervous system: a summary. Neuro Oncol. 2021 Aug 2;23(8):1231-51.
3. Franceschi E, Hofer S, Brandes AA, et al. EANO-EURACAN clinical practice guideline for diagnosis, treatment, and follow-up of post-pubertal and adult patients with medulloblastoma. Lancet Oncol. 2019 Dec;20(12):e715-28.