Chordomas: A Review

Abstract

Chordomas (CHs) are tumors with benign histopathology but exhibit aggressive clinical behavior with invasive and metastatic potential. CHs are challenging tumors to treat, and there are still many questions especially about their optimum treatment. Radical resection at first presentation should be applied on all CHs. Complementary proton therapy irrespective of the quality of resection is now routinely proposed even if its efficacy is not clearly demonstrated in case of incomplete resection. The future of CH treatment is certainly related to a better understanding of the molecular biology and oncogenesis of CHs and consequently to the development of efficient targeted chemotherapies.

Keywords: Chordomas; Endonasal approach; Proton beam therapy.