Chordoma
Chordoma is a
type of cancerous (malignant) tumor that can occur in the bones of the spine
and base of the skull. It is very rare, accounting for only about 1% of all
malignant bone tumors.
Although slow-growing, chordomas can
be difficult to treat because of their location near vital structures such as
the spinal cord and brain. In most cases, treatment for the tumors involves a
combination of surgery and radiation therapy
Cause
Chordomas occur spontaneously. They are not known to be
caused by trauma, environmental factors, or diet. Chordomas are not inherited.
They are not associated with any medical conditions or with the use of any
medications or supplements.
Symptoms
Because chordomas are slow-growing, patients often have
symptoms for a long time—in some cases, more than a year—before they seek
medical attention.
Symptoms depend on the location of the tumor:
- Tumors
in the skull can cause headaches or vision problems.
- Tumors
in the spine can cause pain in the area of the tumor (neck, back, or
tailbone). A tumor pressing on nearby nerves can cause symptoms similar to
a "slipped" or herniated disk. These symptoms may include pain,
weakness, numbness, clumsiness, or tingling in the arms or legs.
- Tumors in the tailbone area can cause a noticeable mass; numbness in the groin area; bowel and bladder problems; or numbness, tingling, and weakness in the legs.
Imaging Studies
Several
imaging studies may be needed to identify the tumor. These may include:
- X-rays
- Computed tomography (CT) scans
- Magnetic resonance imaging
(MRI) scans
While chordomas themselves do not show up well on x-rays, an
x-ray may reveal bone damage caused by the tumor. X-rays of the sacrum are often
difficult for doctors to interpret.
An MRI is considered the best test for determining the total
size of the tumor.
Biopsy
A
biopsy is necessary to confirm a diagnosis of chordoma. In a biopsy, a tissue
sample of the tumor is taken and examined under a microscope.
Staging
Once
the tumor has been identified as chordoma, your doctor will conduct more tests
to determine whether the cancer has spread. This process is known as
"staging." These additional tests may include:
·
Blood
tests
·
CT
scan of the lungs
·
Bone
scan
The part of the body where the first tumor develops is
called the "primary" site. Any parts of the body where it has spread
are called "metastatic" sites.
By identifying the stage of the tumor, your doctor can
determine the most effective treatment strategy.
Treatment
Because of their proximity to the brain and spinal cord,
chordomas are very difficult to treat.
Traditional chemotherapy is not usually very effective and
standard radiation alone rarely cures the tumor.
Whenever possible, surgery is the preferred treatment
option. Surgery is often combined with radiation to treat chordomas. If a tumor
cannot be surgically removed, radiation therapy may help stop the growth of the
tumor. Newer radiation techniques—such as proton beam therapy—may be more
effective than standard radiation at stopping the tumor from growing.
Although it is the preferred treatment option, surgery can
often be difficult to perform because of the important structures that are
located near the tumor. To ensure the best outcome, the surgeon must remove the
tumor and a portion of normal tissue surrounding it. This often results in some
loss of neurologic function. For example, removing tumors in the sacrum may
result in the loss of bowel and bladder control (incontinence), lower extremity
weakness, or loss of sensation in the groin area. It may also cause instability
of the pelvic bones. Your doctor will talk with you about the risks in your
specific case.
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