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01-01-2012 | Review

Current therapeutic options and novel molecular markers in skull base chordomas

Published in: Neurosurgical Review | Issue 1/2012

Abstract

Chordomas are extremely rare tumours. They arise in the spheno-occipital region in 35% of cases. Chordomas usually present benign histopathological features but often exhibit a malignant clinical behaviour. Radical surgical removal and high-dose radiation therapy seem to be effective in tumour control and to improve survival rate. Despite the advancements in microsurgical techniques and the development of radiation therapies, clival chordomas still represent a challenge. Nevertheless it appears that chordomas that have been resected to the same extent and that received post-operative radiotherapy might exhibit different rates of regrowth. This result supports the hypothesis that the recurrence rate of chordomas might be dependent on biological variables other than the extent of resection and the post-operative radiotherapy. Genetic and molecular studies on oncogenesis of chordomas are still limited, but they represent the basis for the development of molecular targeted therapies. We present a review of the current knowledge about skull base chordomas biology, therapeutic options and related clinical outcome.

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Title: Current therapeutic options and novel molecular markers in skull base chordomas
Publication date: 01-01-2012
Published in: Neurosurgical Review / Issue 1/2012
Print ISSN: 0344-5607
Electronic ISSN: 1437-2320
DOI: https://doi.org/10.1007/s10143-011-0354-1

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