Chordoma

Overview for Chordoma:

• Chordomas are complicated tumors to treat because of the involvement of adjacent critical structures such as the brain stem (with clival chordomas), vertebral bodies and spinal cord (with cervical, thoracic and lumbar chordomas), and important nerves that control bowel, bladder and sexual function (with sacral chordomas).
• The tumors are generally slow-growing and can affect adjacent organs, tissues and bones.
• Chordomas may develop at any age but usually occur between 40 and 70 years of age.
• About 300 patients are diagnosed with chordoma each year in the United States, and about 700 in all of Europe.

Presentation

• Skull base: double vision, headaches, cranial nerve palsy (numbness, drooping of face)
• Spine: pain, neurologic symptoms (numbness, upper and lower extremity weakness)
• Sacrum: pain, constipation, stool and bladder incontinence, erectile dysfunction

Imaging

• X-rays show a destructive/lytic lesion (Lytic lesions are areas where bone has been destroyed, leaving a hole in the bone.).
• Usually extends into soft tissue forming a sizable, well-defined mass.
• Additional imaging with MRI or CT helps identify extent of the tumor

Determining Diagnosis:

• Bone biopsy with conscious sedation done by our musculoskeletal radiologist.
• Initial staging scans (e.g. MRI and total spine MRI, chest-abdomen-pelvis CTs) to determine if the disease is localized or has spread (metastasized).
• Our sarcoma/chordoma pathologists confirm diagnosis, which may take up to 1-2 weeks due to the specialized testing of tissue. Malignant bone tumors are graded 1 (low), 2 (intermediate), 3 (high), depending on how aggressive the tumor is.
• There are four subtypes of chordoma:
  • Conventional (or classic) chordoma is the most common form.
  • Poorly differentiated chordoma can be more aggressive and faster growing than conventional chordoma. It is more common in pediatric and young adult patients, and those with skull base (clival) and cervical patients.
  • Dedifferentiated chordoma is the most aggressive and fastest growing chordoma. It is more likely to metastasize than conventional chordoma.
  • Chondroid chordoma is a term used in the past when it was difficult for pathologists to distinguish conventional chordoma from chondrosarcoma (malignant bone tumor of cartilage tissue). This is no longer a problem because nearly all conventional chordomas express brachyury, unlike cartilage tumors like chondrosarcoma that do not express brachyury. Brachyury is a protein, which regulates other genes that are important in embryonic development. Brachyury is highly expressed in nearly every chordoma.

Personalized Treatment Plan:

• Once the diagnosis is confirmed and all scans are reviewed, you will have a lengthy visit with your chordoma team to discuss all treatment options. With your team, you will decide which treatment option is suitable for you, your quality of life and your family.
  1. Preoperative radiation + surgery + postoperative radiation
     • Daily radiation (Monday-Friday) for five weeks.
     • Treatment break for one month to allow radiated skin to recover.
     • 1-3 scheduled procedures are necessary to remove the tumor in total and reconstruct the defect left behind - called "staged surgeries."
       • Reconstruction of the defect can involve use of a metal cage, allograft strut, vascularized fibula, spinal instrumentation and various types of muscle flaps.
     • Postoperative daily radiation once surgical incisions have healed.
  2. Definitive Radiation (which means radiation only)
     • Daily radiation (Monday-Friday) for seven - eight weeks.
• Surveillance: After the initial postop visits (typically at 2 and 6 weeks), routine surveillance is for 10 years with imaging:
  • Every 3 months for 2 years
  • Then every 6 months for 3 years, which equals 5 years of follow-up
  • Annual visit thereafter for 5 more years, which concludes the surveillance standard of care of 10 years (but most patients like to continue with an annual visit)

Read more in our Chordoma booklet.