Treatment of endolymphatic sac tumour (Papillary adenocarcinoma) of the temporal bone

Abstract Aim 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. Materials and 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. Conclusion 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.