Chordoma of the sacrum and mobile spine: a narrative review.
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.
Duke Scholars
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Related Subject Headings
- Treatment Outcome
- Spinal Neoplasms
- Sacrum
- Orthopedics
- Neoplasm Recurrence, Local
- Humans
- Chordoma
- 4201 Allied health and rehabilitation science
- 3202 Clinical sciences
- 1109 Neurosciences
Citation
Published In
DOI
EISSN
Publication Date
Volume
Issue
Start / End Page
Location
Related Subject Headings
- Treatment Outcome
- Spinal Neoplasms
- Sacrum
- Orthopedics
- Neoplasm Recurrence, Local
- Humans
- Chordoma
- 4201 Allied health and rehabilitation science
- 3202 Clinical sciences
- 1109 Neurosciences