title: Management of Locally Recurrent Chordoma of the Mobile Spine and Sacrum: A Systematic Review.
abstract: METHODS
  Systematic review.
  
  
  OBJECTIVE
  To determine evidence-based guidelines for the management of locally recurrent spinal chordoma.
  
  
  BACKGROUND
  Chordoma of the spine is a low-grade malignant tumor with a strong propensity for local recurrence. Salvage therapy is challenging due to its relentless nature and refractoriness to adjuvant therapies. There are currently no guidelines regarding the best management of recurrent chordoma.
  
  
  METHODS
  We combined the results of a systematic review with expert opinion to address the following research questions: (1) For locally recurrent chordoma of the spine without systemic disease, if surgery is planned, should en bloc resection be attempted if technically feasible with acceptable morbidity? (2) For locally recurrent chordoma without systemic disease, in which wide en bloc excision is not possible, what is the treatment of choice? (2) Should adjuvant or neoadjuvant radiation therapy be used in the treatment of locally recurrent chordoma?
  
  
  RESULTS
  A total of nine surgical and seven radiation therapy articles met study criteria. Evidence quality was low or very low. Recurrent disease is associated with predominantly poor outcome, regardless of treatment modality. As for primary chordoma, resection with wide margins appears to confer an advantage with respect to local control, although this effect is attenuated in the setting of relapse. Postoperative radiation therapy likely reduces the rate of further relapse.
  
  
  CONCLUSIONS
  (1) For locally recurrent chordoma of the spine without systemic disease, when surgery is planned, wide en bloc resection should be performed if technically feasible with acceptable morbidity. Strong recommendation, Low Quality of Evidence. (2) For locally recurrent chordoma without systemic disease, in which wide en bloc excision is not possible, partial resection is the treatment of choice. Weak recommendation, Very Low Quality of Evidence. (3) For the treatment of locally recurrent chordoma, high-dose conformal radiation therapy should be administered postoperatively to reduce the risk of further recurrence, and may be considered as a primary therapy. Strong recommendation, Very Low Quality of Evidence.
  
  
  METHODS
  2.
authors.affiliation: Division of Spine, Department of Orthopaedics, University of British Columbia and Vancouver General Hospital, Vancouver, BC, Canada.
authors.forename: Tamir
authors.initials: T
authors.lastname: Ailon
authors.affiliation: Department of Neurosurgery, Rhode Island Hospital, The Warren Alpert Medical School of Brown University, Providence, RI.
authors.forename: Radmehr
authors.initials: R
authors.lastname: Torabi
authors.affiliation: Division of Spine, Department of Orthopaedics, University of British Columbia and Vancouver General Hospital, Vancouver, BC, Canada.
authors.forename: Charles G
authors.initials: CG
authors.lastname: Fisher
authors.affiliation: Department of Neurosurgery, Division of Surgery, The University of Texas MD Anderson Cancer Center, Houston, TX.
authors.forename: Laurence D
authors.initials: LD
authors.lastname: Rhines
authors.affiliation: Department of Neurologic Surgery, Mayo Clinic, Rochester, MN.
authors.forename: Michelle J
authors.initials: MJ
authors.lastname: Clarke
authors.affiliation: Department of Neurosurgery, The John Hopkins University School of Medicine, Baltimore, MD.
authors.forename: Chetan
authors.initials: C
authors.lastname: Bettegowda
authors.affiliation: Department of Degenerative and Oncological Spine Surgery, Rizzoli Institute Bologna, Bologna, Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Boriani
authors.affiliation: Department of Radiation Oncology, Memorial Sloan Kettering Cancer Center, New York, NY.
authors.forename: Yoshiya J
authors.initials: YJ
authors.lastname: Yamada
authors.affiliation: Department of Orthopaedic Surgery, Kanazawa Medical University, Uchinada, Japan.
authors.forename: Norio
authors.initials: N
authors.lastname: Kawahara
authors.affiliation: National Center for Spinal Disorders, Budapest, Hungary.
authors.forename: Peter P
authors.initials: PP
authors.lastname: Varga
authors.affiliation: Department of Neurosurgery, Massachusetts General Hospital, Harvard University, Boston, MA.
authors.forename: John H
authors.initials: JH
authors.lastname: Shin
authors.affiliation: Department of Radiation Oncology, Sunnybrook Odette Cancer Centre, University of Toronto, Toronto, ON, Canada.
authors.forename: Arjun
authors.initials: A
authors.lastname: Saghal
authors.affiliation: Department of Neurosurgery, Rhode Island Hospital, The Warren Alpert Medical School of Brown University, Providence, RI.
authors.forename: Ziya L
authors.initials: ZL
authors.lastname: Gokaslan
mesh_terms: D002817:Chordoma / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D006801:Humans; D009364:Neoplasm Recurrence, Local / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D012447:Sacrum / Q000601:surgery*; D013125:Spinal Neoplasms / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D016896:Treatment Outcome