title: Chordoma.
abstract: OBJECTIVE
  To review developments in chordoma treatment.
  
  
  RESULTS
  Recent series with prolonged follow-up show that adequate margins are necessary for surgery to be curative. Safe margins are often difficult to obtain due to the anatomical sites of chordoma: sacrum, skull base and spine. Tumors in these sites are problematic for radiation therapy as well, and this adds to the need for high doses. Hadrons have therefore been used in addition to, or instead of, photons. New photon beam techniques, e.g. intensity modulated radiation therapy and stereotactic procedures, have recently been evaluated. Although less available than photons, hadrons possess certain advantages. While chemotherapy is poorly active, recent interest has focused on molecular-targeted agents. Imatinib was shown to be active, providing mainly nondimensional tissue responses in a significant proportion of patients, which may improve symptoms and progression-free interval. Epidermal growth factor receptor targeting, anti-angiogenics, and the combination of targeted agents with chemotherapy and radiation therapy are also under scrutiny.
  
  
  CONCLUSIONS
  Two major issues about local treatment remain unresolved: when to complement surgery with radiation therapy, and how best to deliver high doses of radiation therapy to the tumor tissue. Regarding systemic treatment, there is ongoing research into how to exploit molecular-targeted therapies.
authors.affiliation: Adult Sarcoma Medical Oncology Unit, Istituto Nazionale dei Tumori, Milano, Italy. paolo.casali@istitutotumori.mi.it
authors.forename: Paolo G
authors.initials: PG
authors.lastname: Casali
authors.forename: Silvia
authors.initials: S
authors.lastname: Stacchiotti
authors.forename: Claudia
authors.initials: C
authors.lastname: Sangalli
authors.forename: Patrizia
authors.initials: P
authors.lastname: Olmi
authors.forename: Alessandro
authors.initials: A
authors.lastname: Gronchi
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use*; D001549:Benzamides; D002817:Chordoma / Q000188:drug therapy* / Q000532:radiotherapy*; D006801:Humans; D000068877:Imatinib Mesylate; D010879:Piperazines / Q000627:therapeutic use*; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use*; D011743:Pyrimidines / Q000627:therapeutic use*; D012445:Sacrococcygeal Region / Q000473:pathology; D019292:Skull Base Neoplasms / Q000188:drug therapy* / Q000532:radiotherapy*; D013125:Spinal Neoplasms / Q000188:drug therapy* / Q000532:radiotherapy*
chemical_list: D000970:Antineoplastic Agents; D001549:Benzamides; D010879:Piperazines; D047428:Protein Kinase Inhibitors; D011743:Pyrimidines; D000068877:Imatinib Mesylate