title: Skull base chordoma mimicking a preauricular neoplasm in a child: clinicopathological features and biological behaviour.
abstract: 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.
authors.affiliation: Department of Oral and Maxillofacial Surgery, Semmelweis University, Budapest, Hungary. suba@szajseb.sote.hu
authors.forename: Zsuzsanna
authors.initials: Z
authors.lastname: Suba
authors.forename: Péter
authors.initials: P
authors.lastname: Hauser
authors.forename: Miklós
authors.initials: M
authors.lastname: Garami
authors.forename: Katalin
authors.initials: K
authors.lastname: Martonffy
authors.forename: György
authors.initials: G
authors.lastname: Szabó
authors.forename: Béla
authors.initials: B
authors.lastname: Szende
authors.forename: Ildikó
authors.initials: I
authors.lastname: Tarján
authors.forename: Katalin
authors.initials: K
authors.lastname: Gábris
authors.forename: József
authors.initials: J
authors.lastname: Barabás
mesh_terms: 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