title: Systemic therapy options for unresectable and metastatic chordomas.
abstract: Chordoma is an exceedingly rare tumor, marked by a slow growth rate. Surgery is the treatment of choice, but the most frequent sites of origin (spine and skull base) make treatment of primary disease challenging. Local relapses affect more than 50% of cases, with a minority of patients being cured by further surgery. Furthermore, metastases occur in at least 20% of patients. For residual or recurrent disease, high-dose radiation therapy may be indicated. Radiation therapy is currently the preferred local treatment when surgery is problematic, exploiting most recent techniques, including proton beams and carbon ions. However, systemic therapy is needed in patients non-amenable to surgery and/or radiation therapy. We reviewed systemic treatments currently available, and targets possibly druggable in the future in this orphan disease.
authors.affiliation: Sarcoma Unit, Department of Cancer Medicine, Fondazione IRCCS Istituto Nazionale Tumori, via Venezian 1, 20133 Milan, Italy. silvia.stacchiotti@istitutotumori.mi.it
authors.forename: Silvia
authors.initials: S
authors.lastname: Stacchiotti
authors.forename: Paolo Giovanni
authors.initials: PG
authors.lastname: Casali
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols; D001859:Bone Neoplasms / Q000628:therapy*; D002817:Chordoma / Q000628:therapy*; D006801:Humans; D058990:Molecular Targeted Therapy; D009364:Neoplasm Recurrence, Local / Q000601:surgery