Skull base chordoma mimicking a preauricular neoplasm in a child: clinicopathological features and biological behaviour.
BACKGROUND
The extreme rarity of chordomas in childhood, the slow growing nature of these tumours and the diverse symptoms may cause many diagnostic problems.


METHODS
A 9-year-old girl presented with an unusual manifestation of a skull base chordoma. The clinical and pathological features were analysed.


RESULTS
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.


CONCLUSIONS
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.
Suba
Zsuzsanna
Z
Department of Oral and Maxillofacial Surgery, Semmelweis University, Budapest, Hungary. suba@szajseb.sote.hu
Hauser
Péter
P
Garami
Miklós
M
Martonffy
Katalin
K
Szabó
György
G
Szende
Béla
B
Tarján
Ildikó
I
Gábris
Katalin
K
Barabás
József
J
D001932:Brain Neoplasms / Q000175:diagnosis; D002648:Child; D002817:Chordoma / Q000175:diagnosis* / Q000556:secondary; D003937:Diagnosis, Differential; D004428:Ear Neoplasms / Q000175:diagnosis*; D004431:Ear, External / Q000473:pathology*; D004433:Earache / Q000175:diagnosis; D005145:Face / Q000294:innervation; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D006987:Hypesthesia / Q000175:diagnosis; D008175:Lung Neoplasms / Q000556:secondary; D008336:Mandibular Diseases / Q000175:diagnosis; D008409:Mastication / Q000502:physiology; D009361:Neoplasm Invasiveness; D019292:Skull Base Neoplasms / Q000175:diagnosis*; D013702:Temporal Lobe / Q000473:pathology