Chordoma: The Quest for Better Treatment Options

摘要: Chordoma is an extremely rare cancer, with incidence of about one case per million persons year in the USA and Europe (about 300 450 cases year, respectively). The estimated median overall survival patients chordoma approximately 6–7 years, yielding a rough estimate prevalence at 2000 3000 Europe. Primary tumor develops along axial spine between clivus sacrum from residual embryonic notochord. Brachyury (T), transcription factor required for normal development, expressed notochord overexpressed almost all chordoma. primary treatment surgical excision wide local margins, when possible. Radiotherapy also plays significant role adjuvant setting surgery not Unfortunately, advanced and/or metastatic setting, where radiation less clear, options are very limited. To date, there have been no randomized, controlled trials that resulted defined agents clinical benefit systemic treatment. This review briefly describes natural history initial focuses on disease potential avenues research may lead to improved future.