Kivonat:
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.
