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Tumor Location

Chordomas can occur anywhere along the spine, from the head to the tailbone. The most common locations are in the clivus (a bone in the middle of the head) (32%) and sacrum or coccyx (vertebrae at the bottom of the spine) (29%). Less frequently, chordomas can occur in the cervical (neck), thoracic (upper back), and lumbar (lower-back) vertebrae of the spine.[1] Extremely rare cases of chordoma occurring away from the spine have been reported in the ribs, legs and feet.[2]

Chordomas occurring in the head are sometimes called brain tumors, however they do not actually form from brain cells. Chordomas grow out of the bone but because of their location in the skull, they often touch, compress, or invade critical nearby structures such as the nerves, arteries, dura and brainstem. In the lower spine chordomas can compress and displace the bowls and rectum.

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Types of Chordoma

There are three histological subtypes of chordoma: conventional (sometimes called classic), chondroid, and dedifferentiated. Chondroid chordomas tend to be less aggressive than conventional chordomas, while dedifferentiated chordomas are more aggressive, faster growing and more likely to metastasize.

Chordomas are often confused with or misdiagnosed as chondrosarcomas, and vice versa.[3] Both types of tumor can occur in the same locations and often look similar under a microscope. Generally, chondrosarcomas tend to be more responsive to radiation and have a better prognosis.[3] Therefore before proceeding with treatment, it is important to get multiple opinions on the pathological diagnosis from experienced pathologists who routinely see both types of tumors.

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Incidence and Prevalence

In the United States, there are around 300 new cases of chordoma diagnosed each year. Based on this statistic, the annual incidence of chordoma is approximately one new case per million people per year.[1] The incidence in Europe appears to be similar, but is unknown in other continents. Chordomas account for about 3% of all bone tumors and about 20% of primary spinal tumors.[4] Chordomas are the most common tumor of the sacrum and cervical spine.

With an average survival of 7 years, the number of people living with chordoma (prevalence) is approximately 7 per million, or about 2100 in the United States.

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Demographics

Chordomas occur in people of all ages, from infants to the elderly. The median age of diagnosis is 49 for skull base chordomas and 69 for sacral chordomas. Skull base chordomas occur more frequently in younger patients, while sacral chordomas are more common later in life. Overall, the ratio of males to females with chordoma is approximately 1.6 to 1. [1]

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Risk Factors

Chordomas are for the most part sporadic tumors: there are no known causes or risk factors. Rarely, families with multiple affected members have been reported, suggesting that a predisposition for chordomas can be inherited from blood relatives. Additionally, chordomas have been reported in families with tuberous sclerosis, and certain types of childhood brain tumors. It is therefore very important to ask blood-relatives of all ages (grandparents, siblings, aunts/uncles, cousins, nieces/nephews, etc) if they have had chordoma, tuberous sclerosis, or any cancer of the brain or spine. Please notify your doctor of any blood-relatives with these conditions. You may also a summary to the Chordoma Foundation.

If two family members are diagnosed with chordoma then all of the blood relatives that the two chordoma patients share in common are also at risk for chordoma, and may already have occult tumors. If two or more of your relatives have been diagnosed with chordoma you should contact the National Cancer Institute's Familial Chordoma Study to learn about their research to find the genes that cause chordomas.

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Signs and Symptoms

The most common signs of chordoma are pain and neurological changes. Skull base chordomas most often cause headache, neck pain, diplopia (double vision), or facial nerve palsy (paralysis of facial muscles). Chordomas of the spine and sacrum can cause changes in bowel and/or bladder function, pain, aching, tingling, numbness, or weakness of the arms and legs. Often sacral chordomas do not cause symptoms until the tumor is quite large and sometimes a lump is the first sign of a sacral chordoma.[5]

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Treatment

Surgery
Currently, surgery is the first-line treatment for chordomas. Complete resection (removing the entire tumor) during the first surgery provides the best chances for local control and long-term survival.[6; 7] To achieve a complete resection, aggressive surgery is often required and can lead to significant complications or side-effects.[8]

The goal of surgery should be to remove as much of the tumor as possible without causing unacceptable harm. Because outcome and prognosis are largely dependent on the success of the initial surgery, before having any operation it is very important to get multiple opinions from surgical teams who have experience treating chordomas on a regular basis. Some medical centers have multidisciplinary teams of experts who review cases and can help create a coordinated treatment plan for patients with chordomas.

Radiotherapy
In many cases, radiation therapy following surgery is recommended and can improve chances of local control and survival.[6; 9] Because chordomas do not grow rapidly, high doses of radiation are required to kill the tumor cells. Chordomas that are close to critical structures (arteries, brain, brainstem, cranial nerves, dura, spinal nerves), often limit the dose of radiation that can be safely delivered to the tumor. In most cases, proton beam radiation can maximize the dose of radiation to the tumor, while sparing adjacent critical structures. Intensity modulated radiation therapy (IMRT), stereotactic radiosurgery (CyberKnife® or Gamma Knife®)[10] and carbon-ion radiotherapy are also sometimes used to treat chordoma.[11]

Chemotherapy
Chordomas are generally resistant to standard cytotoxic chemotherapy agents, and there are no drugs approved to treat chordoma. Dedifferentiated chordomas are sometimes treated with chemotherapy to kill rapidly multiplying cells.[12] Recently, Gleevec® (imatinib) has been shown to have antitumor effects in some patients with advanced chordomas.[13] Additionally, off-label use of other targeted therapies such as ERBITUX® (cetixumab), Iressa® (gefinitinib), Tarceva® (erlotinib) have been reported.[14]

See the clinical trials section for a list of Phase II clinical trials open to chordoma patients. In general, chordoma patients are eligible for many Phase I sarcoma clinical trials.

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Prognosis and Survival

Chordomas are malignant and potentially life threatening tumors. Currently the median survival in the United States is about 7 years.[1] Advances in surgery, radiation and imaging techniques in the past two decades have increased survival rates and improved chances of a cure.

The overall survival rates are 68% at 5 years and 40% at 10 years.[1] Complete surgical resection offers the best chance for long-term survival.[15; 16] In many cases, radiation therapy can also increase local control rates and prolong survival.

Even after surgery and/or radiation, chordomas tend to return locally - in the same location or in the areas around the original tumor. Many patients undergo multiple surgeries over several years to treat local recurrences. After a local recurrence the chances of achieving a cure are significantly diminished.[17; 18] Distant metastasis (spreading to other body parts) occurs in 20-40% of patients with chordomas of the spine [19; 20] and less than 10% of patients with skull-base tumors.[15] The most common sites of distant metastasis are the lungs, liver, bones, and skin.

It is important to realize that the prognosis for each person is different depending on age, size and location of the tumor, histological subtype and method of treatment. Only your doctor(s) can tell you what your prognosis and risks are.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions.

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