Skull Base Chordoma
A chordoma is a form of bone cancer that can occur anywhere along the length of the
spine, from the base of the skull to the lower back. When it occurs at the base of
the skull, it is known as skull base chordoma. Because of its location in the base
of the skull, it commonly affects vital structures, such as the nerves that control
movement of the face, eyes, and swallowing.
Facts about skull base chordoma
Chordomas are rare cancers. Anyone can develop a chordoma at any age,but they are
more common in older adults.
Types of skull base chordoma
Skull base chordomas are divided into 3 types:
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Conventional. This is the most common type of chordoma and is sometimes called a classic chordoma.
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Chondroid. This type of chordoma has about the same outlook as conventional chordomas.
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Dedifferentiated. The dedifferentiated form of chordoma is more aggressive and grows more quickly than
the other types.
Symptoms
If you have a skull base chordoma, the most common symptoms that you will experience
are related to pain or changes in nerve function. These can include:
Diagnosis
Techniques typically used to diagnose cancer will be used to identify a skull base
chordoma. These may include various scans, such as X-rays, CT scans or MRI, a blood
test, or a biopsy. Testing may be done by a specialist, such as a neurologist or a
neurosurgeon.
One common misdiagnosis that can occur with a chordoma is confusing it with a chondrosarcoma,
another type of bone tumor. Both types of tumors can occur in similar locations in
the body, and they look similar as well. Chondrosarcomas tend to respond better to
radiation and have a better outlook.
Treatment
The primary treatment for a skull base chordoma is surgery to remove as much of the
tumor as possible. This is often an aggressive procedure that can lead to some side
effects, but it offers the best chance for long-term survival. You and your health
care team should weigh the risks and benefits of the procedure before the surgery.
Once the surgery is complete, an aggressive course of radiation therapy is used to
kill the surrounding tumor cells. Proton beam radiation is often used because it can
focus on the needed area without exposing any critical organs and structures around
it to radiation. Proton beam radiation offers the advantage of increasing the tumor
dose while minimizing the dose to the adjacent normal tissue. Other forms of radiation
are sometimes used as well.
Chemotherapy is typically not effective in treating a skull base chordoma. Currently,
no drugs are approved for treating chordomas, but some have shown effectiveness in
clinical trials.
Prevention
Skull base chordomas are rare disorders, and it is not known how to prevent them.
Managing skull base chordomas
A skull base chordoma is a malignant and potentially life-threatening tumor. People
need to be prepared for a difficult fight against the illness, one that will involve
challenging treatments, such as surgery and radiation therapy.
Outcomes from chordoma treatment have improved in recent decades. Still, it's best
to address potential end-of-life issues as part of a skull base chordoma management
plan.