Introduction: The extreme rarity of chordomas in childhood, the slow growing nature of these tumours and the diverse symptoms may cause many diagnostic problems.
Patient: A 9-year-old girl presented with an unusual manifestation of a skull base chordoma. The clinical and pathological features were analysed.
Result: In the present case, the initial symptoms of the skull base tumour were completely misleading. The otodynia, the masticatory difficulties and the mass in the preauricular region were not characteristic of skull base chordomas. The female sex, the young age, the large tumour size and the atypical histological pattern of the tumour all indicated a very poor prognosis.
Conclusion: The rarity of this tumour in childhood and the atypical lateral and intracranial spread resulted in a serious delay of the diagnosis and in a fatal outcome.
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