Cancer is always frightening, no matter where it occurs. One type of cancer that is particularly distressing is chordoma, a rare malignant bone tumor that forms in the spine or the base of the skull. More specifically, it’s a type of sarcoma, which is a broad group of cancers that begin in the bones and your soft (connective) tissues. A chordoma tumor usually grows slowly, often without symptoms at first, and then might cause symptoms for years before doctors find it. Chordoma can be fatal, but with early diagnosis and appropriate treatment, many chordoma patients can live for a decade or more and some can be cured. That’s why it is so crucial to find skilled surgeons with extensive experience treating chordomas like the first-class experts you’ll find at OCC – Advanced Orthopedic & Sports Medicine Specialists in Denver, Parker, or Aurora, Colorado.

OVERVIEW

Chordomas account for about 3 percent of all bone tumors and about 20 percent of primary spinal tumors. They are diagnosed in just 1 in 1 million people every year. There’s nothing that can be done to prevent developing chordoma. Most cases happen randomly. They develop in anyone at any age, but they are most likely to occur in adults aged 40 to 80. Men and people assigned male at birth are approximately 1.5 times as likely to have a chordoma as women and people assigned female at birth. However, chordomas, especially the types that appear at the bottom of the spine, affect more women than men. Skull base chordomas are more common in younger patients; spinal chordomas are more likely after age 50. It is possible to develop more than one chordoma tumor at a time, in different places along the spine.

WHAT IS A CHORDOMA?

Chordomas are complicated tumors to treat due to the involvement of critical structures such as the brainstem, spinal cord, and important nerves and arteries. They can also come back, or recur, after treatment — usually in the same place as the first tumor. This is called a local recurrence. In about 30 to 40 percent of patients, the tumor eventually spreads, or metastasizes, to other parts of the body, usually to the lungs. After it has recurred, it can cause death, typically due to tissue destruction in the spinal cord, or brainstem. Different segments of the spine correlate with different parts of the body, and so a chordoma’s effects depend on where along the spine it occurs:

• The skull base includes a bone called the clivus, where about a third of all chordomas develop. Tumors there affect cranial and facial nerves and can affect vision.
• The cervical spine starts just below the skull and ends just above the thoracic spine. Its nerves branch off to the arms and upper chest.
• The thoracic spine is the mid-back. Its nerves branch off to the abdomen and chest.
• The lumbar spine is the lower back. Its nerves branch off to the hips, knees, ankles, and toes.
• The sacrum is the base of the spine and below that is the coccyx. A chordoma in this area can affect the function of the legs, pelvis, bowel, and bladder.

TYPES OF CHORDOMA

The World Health Organization (WHO) recognizes three distinct types of chordoma based on what the cells look like under a microscope (their histology):

• Classic/conventional chordoma. This is the most common type (80-90% of all cases). It’s made up of a unique type of cell that looks “bubbly”.
• Chrondoid is a variant of this type. Under a microscope, it has certain features of a conventional chordoma and a chondrosarcoma, which is another type of cancer, but one that derives from cells that produce cartilage.
• Differentiated chordoma is the rarest (less than 5% of cases). It’s more aggressive, faster growing, and more likely to metastasize.

CAUSES

Chordomas form from the left-over cells that were important in the development of the spine before birth. These cells are called notochord cells. When the notochord cells don’t disappear after birth, they can turn into chordomas. The vast majority of people with chordoma have no other family members with this cancer. However, a small number of families with multiple relatives with chordoma have been reported worldwide. In most of them, chordoma has occurred in a parent and one or more of his/her children. Researchers continue to investigate the question of chordoma, but finding answers is challenging because chordomas are so rare.

SYMPTOMS

The most common signs of chordoma are pain and neurological changes. Skull base chordomas most often cause headaches, facial numbness/pain, neck pain, blurry vision, or double vision. If large enough, they may affect facial sensation or movement, voice, speech, and swallowing function. Chordomas of the mobile 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.

NON-SURGICAL TREATMENTS

If surgery isn’t an option, the following non-surgical treatments may be recommended instead:

• Radiation therapy. This uses high-energy beams such as X-rays to kill cancer cells. It may also be used before surgery to shrink a cancer and make it easier to remove. Treatment with newer types of radiation treatment such as proton therapy allows doctors to use higher doses of radiation while protecting healthy tissue which may be more effective in treating a chordoma.
• Radiosurgery. Stereotactic radiosurgery uses multiple beams of radiation to kill the cancer cells in a very small area. Each beam isn’t very powerful, but the point where all the beams meet—at the chordoma—receives a large dose of radiation to kill the cancer cells.
• Target therapy. Targeted therapy uses drugs that focus on specific abnormalities within cancer cells. By attacking these abnormalities, this can cause cancer cells to die. Targeted therapy is sometimes used to treat chordoma that spreads to other areas of the body.

WHEN IS SURGERY INDICATED?

Some scenarios where surgery might be considered include:

• Symptomatic tumors where the chordoma is causing severe pain, neurological deficits like weakness, numbness, or tingling, or problems with organ function due to compression or invasion by the tumor.
• Tumor size and location. Large tumors or those located in critical areas are likely to cause significant trauma if left untreated.
• Failed conservative treatment. When other treatments like radiation therapy or chemotherapy have been ineffective in controlling the tumor’s growth symptoms.
• Risk of complications. If there is a risk of complications from the tumor’s growth such as spinal cord compression or organ dysfunction, surgery may be needed to prevent further damage.

Depending on the patient’s overall health and treatment goals, surgery may be pursued to remove as much of the tumor as safely as possible to relieve symptoms and improve quality of life.

GETTING THE RIGHT DIAGNOSIS. GETTING THE RIGHT DOCTOR.

Getting an accurate diagnosis and immediate treatment for chordoma can make all the difference in how quickly chordomas grow, the extent to which they can metastasize, the rate of recurrence, the neurological involvement, and most importantly, survival rates. It is important to remember that the prognosis for each person is unique. Because of its rarity, diagnosis and treatment for chordoma is an intricate process and you need the most highly skilled doctors with unmatched experience such as the first-class surgeons at Advanced Orthopedics in Denver, Parker, or Aurora, Colorado. From the moment a chordoma may be suspected, the caring and understanding specialists at Advanced Orthopedics will be there for you, to reassure you and answer all your questions. After a thorough exam, imaging studies will be ordered. X-rays can show abnormalities in the bones and sometimes reveal a mass or tumor. CT scans provide detailed cross-sectional images of the affected area, which can help visualize the extent and location of the tumor. MRIs are extremely useful for imaging soft tissues and can provide detailed images of the spinal cord, nerve roots, and surrounding structures. They are particularly effective in visualizing chordomas. The only way your surgeon can really diagnose a chordoma is with a needle biopsy. Determining how the biopsy should be performed requires careful planning by your medical team. Doctors need to perform the biopsy in a way that won’t interfere with future surgery to remove the cancer. You won’t be alone anywhere in the entire process. Not only will you get the best care throughout, the other thing you’ll get at Advanced Orthopedics, is hope. Schedule an appointment today.