Chordoma in Children

Chordoma is a very rare type of bone tumor that forms anywhere along the spine from the base of the skull to the tailbone. In children and adolescents, chordomas develop more often in the base of the skull, making them hard to remove completely with surgery.

Childhood chordoma is linked to the condition tuberous sclerosis, a genetic disorder in which tumors that are benign (not cancer) form in the kidneys, brain, eyes, heart, lungs, and skin.

Chordoma may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:

• Headache
• Neck or back pain
• Double vision
• Paralysis of the muscles in the face
• Numbness, tingling, or weakness of the arms and legs
• A change in bowel or bladder habits

Other conditions that are not chordoma may cause these same signs and symptoms. Chordomas may recur (come back), usually in the same place, but sometimes they recur in other areas of bone or in the lungs.

Treatment of chordoma in children may include the following:

• Surgery to remove as much of the tumor as possible, followed by radiation therapy.
• Proton beam radiation therapy may be used.

Treatment of recurrent chordoma in children may include the following:

• A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.