Chordoma of the sacrum and mobile spine: a narrative review

Zach Pennington, Jeff Ehresman, Edward F. McCarthy, A. Karim Ahmed, Patricia D. Pittman, Daniel Lubelski, C. Rory Goodwin, Daniel M. Sciubba

Research output: Contribution to journalReview articlepeer-review

Abstract

Chordoma is a notochord-derived primary tumor of the skull base and vertebral column known to affect 0.08 to 0.5 per 100,000 persons worldwide. Patients commonly present with mechanical, midline pain with or without radicular features secondary to nerve root compression. Management of these lesions has classically revolved around oncologic resection, defined by en bloc resection of the lesion with negative margins as this was found to significantly improve both local control and overall survival. With advancement in radiation modalities, namely the increased availability of focused photon therapy and proton beam radiation, high-dose (>50 Gy) neoadjuvant or adjuvant radiotherapy is also becoming a standard of care. At present chemotherapy does not appear to have a role, but ongoing investigations into the ontogeny and molecular pathophysiology of chordoma promise to identify therapeutic targets that may further alter this paradigm. In this narrative review we describe the epidemiology, histopathology, diagnosis, and treatment of chordoma.

Original languageEnglish (US)
Pages (from-to)500-517
Number of pages18
JournalSpine Journal
Volume21
Issue number3
DOIs
StatePublished - Mar 2021

Keywords

  • Chordoma
  • Clinical guidelines
  • En bloc resection
  • Evidence-based medicine
  • Overall survival
  • Proton beam therapy
  • Stereotactic radiation

ASJC Scopus subject areas

  • Surgery
  • Orthopedics and Sports Medicine
  • Clinical Neurology

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