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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.

Facts about skull base chordoma

Chordomas are rare cancers. According to the Chordoma Foundation, on average, only one in 1 million people is affected each year, or about 300 new cases in the U.S. annually. Although anyone can develop a chordoma at any age, a skull base chordoma is diagnosed most often around age 49, and in men more often than in women.

Types of skull base chordoma

Skull base chordomas are divided into three types:

  • Conventional. This is the most common type of chordoma and is sometimes called a "classic" chordoma. It is more aggressive than a chondroid chordoma, but less aggressive than dedifferentiated.

  • Chondroid. This is the least aggressive type of chordoma. 

  • 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:

  • Headache

  • Neck pain

  • Double vision

  • Paralysis of the facial muscles

  • Speech changes

  • Swallowing problems

Diagnosis

Techniques typically used to diagnose cancer will be used to identify a skull base chordoma. These may include various scans, such as 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. 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 leads 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. 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. These drugs include Gleevec (imatinib), Erbitux (cetuximab), Iressa (gefitinib), and Tarceva (erlotinib).

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. Patients need to be prepared for a difficult fight against the illness, one that will involve challenging treatments, such as surgery and radiation therapy.

The average life expectancy for someone with a chordoma is about seven years. Survival rates at five years are 58 percent, and 40 percent at 10 years. 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. 

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