These are malignant tumours, arising from undeveloped stem cells either in the portion of the brain that controls voluntary movement, the cerebellum (medullobalstoma) or elsewhere within the brain (Primitive Neuroectodermal Tumour, PNET). These tumours can spread to other parts of the brain and spinal cord. Their treatment involves maximum possible surgical removal of the tumour by experienced neurosurgeons. Following surgery and within a few weeks after recovery, depending on the patient's age, location of the tumour, whether there is any residual tumour following surgery or disseminated disease, the treatment can include higher or lower doses of radiation therapy to the brain and spine and/or several cycles of intensive chemotherapy (Cisplatin, Cyclophosphamide, Vincristine, CCNU, retinoic acid) for anywhere between 8 months to a year. Radiation therapy to the brain and spine is avoided if possible in young children due to the potential for neuro-cognitive damage (memory, IQ, concentration ,etc..). Overall, the prognosis is good for medulloblastoma and fair for PNET.

Patients who experience relapse (re-growth of their tumour) unfortunately have a poor prognosis and their treatment options could include, depending on prior therapy and the pattern of relapse, surgery, irradiation, high doses of chemotherapy, novel chemotherapy combinations or enrollment on clinical trials with experimental drugs.