Approach Considerations

Treatment options include the following:

Low-dose ^[25]or high-voltage radiation therapy
Combined radiation and surgery
Surgical excision alone ^[26]

It has been generally accepted that complete surgical excision of the tumor is the only curative procedure. Because of local invasion, many tumors (especially skull-base chordomas) may not be amenable to complete surgical excision, and the local recurrence rate is high.^{[27, 28, 29, 30, 31, 32]} Sterotactic radiosurgery (SRS) may be effective for some chordomas.^[33]

Surgical Care

Tumors detected and diagnosed early have a favorable prognosis if treated with a complete or en-bloc excision.^[34] A complete excision may involve a combined anterior-posterior operation, with anterior vertebrectomy, strut grafting, and possible stabilization with instrumentation. This is followed by a posterior decompression that also removes the pedicles and by stabilization of the spine with instrumentation above and below the excised levels.

Extension of the tumor beyond the confines of the vertebral body decreases the chances of a complete cure. Resection of the psoas muscle and sacrifice of lumbar or sacral nerve roots may be necessary. Preservation of both S3 nerve roots is required if normal bladder and bowel function are to be expected postoperatively. In these cases, surgical margin decisions are made on an individual basis. If complete resection of the tumor cannot be carried out and residual tumor is present either in bone or soft tissue, postoperative megavoltage radiation therapy is indicated.

The local recurrence rate is affected by tumor contamination of the surgical wound.^[35] In one study, the recurrence rate was 64% when contamination was present and 28% when no contamination was present.^[36]

A systematic review by Bin-Alamer et al assessed SRS with or without postoperative fractionated radiation therapy in adults with skull-base chordomas.^[33] Tumor control and survival rates for SRS alone were not found to be inferior to those for SRS plus fractionated radiation therapy.

The endoscopic endonasal approach is ain increasingly popular option for for clival chordomas.^[37]

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Media Gallery

MRI scan demonstrates an osteolytic lesion involving the vertebral body. This lesion occurred in the mobile vertebral column.
Histologic section demonstrating lobular arrangement of tumor cells surrounded by fibrous connective tissue septa. Cellularity is variable with cells present in a pale myxoid matrix.
S-100 immunostain of physaliphorous cells demonstrating strong nuclear staining.
Cytologic preparation demonstrating characteristic physaliphorous cells with bubbly cytoplasm at higher magnification.
Cytologic preparation demonstrating a sheet of low-grade polygonal large cells in a background of pale myxoid matrix.

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Author

Nagarjun Rao, MD, FRCPath Pathologist, Great Lakes Pathologists, Aurora Clinical Laboratories

Nagarjun Rao, MD, FRCPath is a member of the following medical societies: American Society for Clinical Pathology, United States and Canadian Academy of Pathology, College of American Pathologists, Royal College of Pathologists

Disclosure: Nothing to disclose.

Coauthor(s)

Vivek Panikkar, MBBS, MS, MCh, FRCS Consulting Surgeon, Departments of Trauma and Orthopedics, Doncaster Royal Infirmary, UK

Disclosure: Nothing to disclose.

Raj Rao, MD Professor of Orthopaedic Surgery and Neurosurgery, Department of Orthopedic Surgery, Medical College of Wisconsin

Raj Rao, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, North American Spine Society

Disclosure: Nothing to disclose.

Vinod B Shidham, MD, FRCPath Professor, Vice-Chair-AP, and Director of Cytopathology, Department of Pathology, Wayne State University School of Medicine, Karmanos Cancer Center and Detroit Medical Center; Co-Editor-in-Chief and Executive Editor, CytoJournal

Vinod B Shidham, MD, FRCPath is a member of the following medical societies: American Association for Cancer Research, American Society of Cytopathology, College of American Pathologists, International Academy of Cytology, Royal College of Pathologists, United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Jeffrey A Goldstein, MD Clinical Professor of Orthopedic Surgery, New York University School of Medicine; Director of Spine Service, Director of Spine Fellowship, Department of Orthopedic Surgery, NYU Hospital for Joint Diseases, NYU Langone Medical Center

Jeffrey A Goldstein, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American College of Surgeons, American Orthopaedic Association, AOSpine, Cervical Spine Research Society, International Society for the Advancement of Spine Surgery, International Society for the Study of the Lumbar Spine, Lumbar Spine Research Society, North American Spine Society, Scoliosis Research Society, Society of Lateral Access Surgery

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for: Medtronic, Nuvasive, NLT Spine, RTI, Magellan Health<br/>Received consulting fee from Medtronic for consulting; Received consulting fee from NuVasive for consulting; Received royalty from Nuvasive for consulting; Received consulting fee from K2M for consulting; Received ownership interest from NuVasive for none.

Additional Contributors

James F Kellam, MD, FRCSC, FACS, FRCS(Ire) Professor, Department of Orthopedic Surgery, University of Texas Medical School at Houston

James F Kellam, MD, FRCSC, FACS, FRCS(Ire) is a member of the following medical societies: American Academy of Orthopaedic Surgeons, Orthopaedic Trauma Association, Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

William O Shaffer, MD Former Orthopedic Spine Surgeon, Northwest Iowa Bone, Joint, and Sports Surgeons

William O Shaffer, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Orthopaedic Association, International Society for the Study of the Lumbar Spine, Kentucky Medical Association, Kentucky Orthopaedic Society, North American Spine Society, Southern Medical Association, Southern Orthopaedic Association

Disclosure: Nothing to disclose.

Chordoma in Orthopedic Surgery Treatment & Management

Approach Considerations

Surgical Care

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