Malignant intracranial chordoma and sarcoma of the clivus in infancy

Neuroradiological and clinical findings suggesting chordoma of the clivus are described in three children. They presented with torticollis as the primary and predominant symptom. Progressive deterioration with development of slurred speech, signs of intracranial pressure and further cranial nerve involvement led to more extensive neuroradiological investigation. The clinical and neuroradiological findings in these three children were similar; the prognosis was unfavourable for two of the children. The histological diagnosis in two cases was malignant chordoma; in the third case it was undifferentiated sarcoma of the clivus.