Chordoma Health Article

Definition

Chordomas are rare tumors of the central nervous system (brain and spinal cord).

Description

Chordomas are slow-growing tumors that invade bone and tissue surrounding the spinal column. They rarely spread to other parts of the body, but they can cause considerable damage or death because they destroy bone and soft tissue and often grow along the roots of nerves, putting pressure on the nerves and disrupting their function.

Chordomas appear at the base of the skull about 60% of the time and in the sacrum, located at the base of the spine, about 30% of the time. The other 10% of chordomas can occur anywhere else along the spinal column.

Demographics

Chordomas are very rare, accounting for between 1-4% of tumors of the brain and spinal column. Chordomas that occur at the base of the skull are most common in adults between 30 and 40 years of age. Those tumors that arise at the sacrum, located at the base of the spine, most commonly appear in older adults between the ages of 50 and 70. Chordomas are about twice as common in men as they are in women.

Causes and symptoms

During the fourth through sixth week of fetal development, a group of cells come together to form a structure called the notochord. The notochord defines the vertical mid-line of the body, and the spinal column develops around it. Normally, as development progresses, the noto-chord degenerates and disappears, except for small bits that become part of the disks between the spinal vertebrae. Chordomas are believed to develop from pieces of noto-chord that, for some reason, do not break down as they should. Over many years, these harmless bits of notochord transform and become malignant, forming chordomas.

Symptoms of chordoma depend on where the tumor is located. They are often vague and similar to symptoms of other tumors or even other conditions. Tumors located at the base of the skull may cause headaches, difficulty swallowing, or seizures depending on how much they have invaded the bones of the skull. Tumors located on the sacrum can cause general low back pain or difficulty with bowel and bladder control.