title: Treatment of endolymphatic sac tumour (Papillary adenocarcinoma) of the temporal bone.
abstract: OBJECTIVE
  To define a better treatment of sporadic endolymphatic sac tumours (ELST) analysing our experience and literature available data.
  
  
  BACKGROUND
  ELST can arise as sporadic case (rare) or as a part of von Hippel-Lindau (VHL) disease. It is a low grade malignancy with local spread by continuity.
  
  
  METHODS
  we described our experience with 7 cases with up to date follow up.
  
  
  RESULTS
  Five cases were free of disease after first surgical procedure. One case had recurrence in the temporal lobe after 12 years. One case had two surgical procedures followed by irradiation and died five years after radiotherapy with a slow disease progression.
  
  
  CONCLUSIONS
  With increasing expertise in the skull base surgery, complete tumour excisions are achieved in majority of the more recent cases and appear to be the treatment of choice. External irradiation is also used as palliative measures with doubtful effectiveness. Some recent reports showed encouraging results with gamma knife radiosurgery.
authors.affiliation: Department of Otorhinolaryngology, Humanitas Research Hospital, Rozzano, Milan, Italy.
authors.forename: Arturo Mario
authors.initials: AM
authors.lastname: Poletti
authors.affiliation: Otorhinolaryngology, School of Medicine and Health Sciences, University of Papua New Guinea, Papua New Guinea.
authors.forename: Siba Prasad
authors.initials: SP
authors.lastname: Dubey
authors.affiliation: Department of Otorhinolaryngology, Humanitas Research Hospital, Rozzano, Milan, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Colombo
authors.affiliation: Department of Otorhinolaryngology, Humanitas Research Hospital, Rozzano, Milan, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Cugini
authors.affiliation: Department of Otorhinolaryngology, Humanitas Research Hospital, Rozzano, Milan, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Mazzoni
keywords: Endolymphatic sac; Papillary adenocarcinoma; Surgery; Temporal bone; Tumour