Current therapeutic options and novel molecular markers in skull base chordomas

Filippo Gagliardi, Nicola Boari, Paola Riva, Pietro Mortini

Research output: Contribution to journalArticle

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.

Original languageEnglish
Pages (from-to)1-13
Number of pages13
JournalNeurosurgical Review
Volume35
Issue number1
DOIs
Publication statusPublished - Jan 2012

Keywords

  • Chemotherapy
  • Molecular markers
  • Radiation therapy
  • Skull base chordomas
  • Skull base surgery

ASJC Scopus subject areas

  • Clinical Neurology
  • Surgery

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