Early morning headache with vomiting in a 5 year old boy

A 5½ year old boy presented with a 10 day history of progressively worsening early morning headache, with vomiting and subsequent development of slurred speech. Before this presentation his development had been normal, with no delay in developmental milestones or abnormality in vision. He was referred to the emergency department for an urgent magnetic resonance imaging (MRI) scan, which showed a tumour arising from the fourth ventricle with a maximum dimension of 3 cm. There was slight dilation of the fourth ventricle, but no hydrocephalus or obvious disease in the spine or leptomeninges.

He underwent craniotomy and gross total excision of the mass. Cytological examination of the cerebrospinal fluid showed no malignant cells. Histological examination of the tumour showed that it was a medulloblastoma, with large pale nuclei (high nuclear to cytoplasmic ratio), cell-cell wrapping, and a high mitotic rate. There were several foci of apoptosis but no sheets of necrosis. Molecular analysis of the tumour showed that the c-Myc and N-Myc genes were amplified. Chemotherapy was started in accordance with Children Cancer and Leukaemia Group guidelines. This involves high dose intensity chemotherapy followed by craniospinal hyperfractionated accelerated radiotherapy.