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

Arturo Mario Poletti; Siba Prasad Dubey; Giovanni Colombo; Giovanni Cugini; Antonio Mazzoni

doi:10.1016/j.rpor.2015.06.003

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

Arturo Mario Poletti, Siba Prasad Dubey, Giovanni Colombo, Giovanni Cugini, Antonio Mazzoni

Istituto Clinico Humanitas

Research output: Contribution to journal › Article › peer-review

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.

Original language	English
Journal	Reports of Practical Oncology and Radiotherapy
DOIs	https://doi.org/10.1016/j.rpor.2015.06.003
Publication status	Accepted/In press - Oct 7 2014

Keywords

Endolymphatic sac
Papillary adenocarcinoma
Surgery
Temporal bone
Tumour

ASJC Scopus subject areas

Cancer Research
Oncology
Radiology Nuclear Medicine and imaging

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title = "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.",

keywords = "Endolymphatic sac, Papillary adenocarcinoma, Surgery, Temporal bone, Tumour",

author = "Poletti, {Arturo Mario} and Dubey, {Siba Prasad} and Giovanni Colombo and Giovanni Cugini and Antonio Mazzoni",

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T1 - Treatment of endolymphatic sac tumour (Papillary adenocarcinoma) of the temporal bone

AU - Poletti, Arturo Mario

AU - Dubey, Siba Prasad

AU - Colombo, Giovanni

AU - Cugini, Giovanni

AU - Mazzoni, Antonio

PY - 2014/10/7

Y1 - 2014/10/7

N2 - 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.

AB - 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.

KW - Endolymphatic sac

KW - Papillary adenocarcinoma

KW - Surgery

KW - Temporal bone

KW - Tumour

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