title: Immunotherapy as a Potential Treatment for Chordoma: a Review.
abstract: Chordoma is a locally aggressive primary malignancy of the axial skeleton. The gold standard for treatment is en bloc resection, with some centers now advocating for the use of radiation to help mitigate the risk of recurrence. Local recurrence is common, and salvaging local failures is quite difficult. Chemotherapy has been ineffective and small molecule targeted therapy has had only marginal benefits in small subsets of patients with rare tumor phenotypes or refractory disease. Recent successes utilizing immunotherapy in a variety of cancers has led to a resurgence of interest in modifying the host immune system to develop new ways to treat tumors. This review will discuss these studies and will highlight the early studies employing immune strategies for the treatment of chordoma.
authors.affiliation: Department of Orthopaedic Surgery, Massachusetts General Hospital Harvard Medical School, 55 Fruit Street Yawkey Building Suite 3A, Boston, MA, 02114-2696, USA.
authors.forename: Shalin S
authors.initials: SS
authors.lastname: Patel
authors.affiliation: Department of Orthopaedic Surgery, Massachusetts General Hospital Harvard Medical School, 55 Fruit Street Yawkey Building Suite 3A, Boston, MA, 02114-2696, USA. jhschwab@partners.org.
authors.forename: Joseph H
authors.initials: JH
authors.lastname: Schwab
mesh_terms: D017951:Antigen Presentation / Q000276:immunology; D000951:Antigens, Neoplasm / Q000276:immunology; D002817:Chordoma / Q000276:immunology / Q000473:pathology / Q000628:therapy*; D006680:HLA Antigens / Q000276:immunology; D006801:Humans; D007107:Immune System; D007167:Immunotherapy; D009364:Neoplasm Recurrence, Local / Q000276:immunology / Q000473:pathology / Q000628:therapy*
chemical_list: D000951:Antigens, Neoplasm; D006680:HLA Antigens
keywords: Antigen processing; Bone tumors; CSPG4; CTLA-4; Carbon-ion; Chimeric antigen receptor; Chordoma; HLA; Immune checkpoint; Immune escape; Immune system; Immunotherapy; MHC; PD-1; PD-L1; PD-L2; Proton; Radiation; Radiotherapy; Sarcoma; Tumor antigen; Vaccine