Chordoma
Chordomas are malignant tumors that can develop anywhere along the spine or at the base of the skull. They most commonly occur in the lower back (sacral area) and the base of the skull, each accounting for approximately one-third to one-half and one-third of cases, respectively. These tumors originate from remnants of the notochord, an embryonic tissue that eventually forms the center of spinal disks.
Characteristics and Behavior
Although chordomas typically grow slowly, they can metastasize and become locally aggressive. Their growth can cause significant problems by putting pressure on or invading critical parts of the brain or spine, leading to pain, nerve issues, or potentially life-threatening complications.
Symptoms
As chordomas grow, they can press on the spine, brain, and nerves, leading to symptoms specific to their location. These symptoms may include pain, tingling, numbness, weakness, lack of bladder or bowel control, sexual dysfunction, vision problems, endocrine issues, and swallowing difficulties. In cases where the chordoma has grown very large, it might be possible to feel a lump.
Causes
The exact cause of cancerous chordomas is unknown. In most cases, doctors do not understand why cancer develops from leftover notochord tissue. Researchers are investigating this question, but finding answers is challenging due to the rarity of chordomas.
Risk Factors
There are no known environmental, dietary, or lifestyle risk factors for chordoma. Most chordomas occur randomly and are not due to inherited genetic traits. However, several genetic factors are associated with chordoma.
- Familial Chordoma: In rare cases, multiple family members may be affected by chordoma, indicating a strong genetic predisposition. Some families with familial chordoma have an extra copy of the brachyury gene, though no test currently exists for detecting these extra copies. The National Cancer Institute is conducting a genetics study to identify additional hereditary causes of chordoma.
- Tuberous Sclerosis Complex (TSC): Children with TSC, a genetic disease caused by changes in the TSC1 or TSC2 genes, have a higher incidence of chordomas.
Treatment Options
Prompt treatment is essential and involves preoperative planning, surgery (en bloc resection), and postoperative therapy. After surgery, patients are closely monitored in the neurosurgical intensive care unit (NCCU) and later undergo physical and occupational therapy. Radiation therapy may also be necessary to reduce tumor size or prevent spread.
Due to the risk of regrowth, consistent follow-up is required. Patients typically need an MRI every three months during the first year post-surgery, with the interval potentially increasing over time.