Chordoma

Read more in our Chordoma booklet.

Chordoma is rare bone tumor believed to come from remnants of embryonic tissue left behind in the sacrum and lower spine. It usually occurs between the ages of 40-70 years, slightly more frequent in males. Sacral chordomas are relentless, slow growing tumors. Patients may have long histories of mild discomfort in the lower spine and tenderness with prolonged sitting. A large soft tissue mass may be felt in the middle of the sacrum or during a rectal examination. Usually neurologic symptoms occur which include constipation because the lesion grows and pushes forward on the rectum; low back pain radiating down the legs because the lesion is pushing against, pinching or engulfing the nerve roots of the sacrum, and loss of sacral nerve sensation causing lower leg weakness, loss of bowel and bladder control, and loss of sexual function because of the the tumor's involvement with the sacral nerves. On x-ray, the chordoma has a typical appearance because of its location in the middle of the sacrum and its destruction of bone with poorly defined margins.

Treatment involves surgical resection of the tumor which is technically difficult due to the location in the sacrum and the proximity (closeness) of vital organs and nerves. Radiation therapy is sometimes given preoperatively, followed by resection with possible intraoperative radiation, internal radiation implants (brachytherapy) postoperatively, and/or proton beam therapy as additional therapy. Chemotherapy has minimal effect on chordomas. Heat ablation has recently been tried for therapy with positive results. Some patients are referred to pain specialists until the neurologic symptoms subside.

Despite the location and difficulty in obtaining complete eradication of a chordoma, most patients have a good long-term survival prognosis. Alterations in life style may be necessary depending upon the effect of neurologic loss from the tumor or treatments. Tumors may recur and require more surgery or radiation. Varying degrees of loss of bowel or bladder control or sexual function can occur, and most patients are able to cope well with this. Occasional patients have persistent pain which can be controlled with a variety of new treatment modalities.