Chondrosarcoma is a rare type of bone tumor found mostly in middle-aged adults. These malignant (cancerous) growths begin in the cells that produce cartilage — the connective tissue that covers the ends of your bones.
These tumors are typically found when cartilage is identified in areas of bone where it is not supposed to be. It can be found in any bone but is commonly identified in the femur (thigh bone), humerus (upper arm), pelvis (hips), and spine (backbone). More rarely, chondrosarcoma may affect the ribs and other areas of the body.
The most common treatment for chondrosarcoma is surgery.
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What Is Chondrosarcoma?
Chondrosarcoma is a rare type of primary bone cancer in which malignant (cancerous) tumors begin in the bone. This type of cancer differs from cancers that spread to the bone from another organ. Chondrosarcoma can sometimes occur in soft tissue (such as muscles) near your bones.
Overall, chondrosarcoma is the second most common type of primary bone cancer, after osteosarcoma. But in adults, it is the most common type of primary bone cancer.
Chondrosarcoma is a malignant tumor of the cells that produce cartilage — the flexible, sturdy tissue that cushions the places where bones meet. Cartilage is found throughout the body and forms structures like your nose and ears.
These tumors can start anywhere in the body but begin most often in the bones of the pelvis, ribs, thighs, and arms. Rarely, they may occur in the skull or spine.
Most chondrosarcomas grow very slowly. They usually occur in people between the ages of 40 and 70. They happen equally in men and women.
Other types of primary bone cancers are:
- Ewing sarcoma — This bone cancer occurs most often in older children and teens, typically in the upper and lower leg, pelvis, upper arm, or ribs, but can be in any bone.
- Osteosarcoma — This aggressive bone cancer occurs in teenagers and young adults, usually in the thighbone or shinbone, but can be in any bone.
How common is chondrosarcoma?
Chondrosarcoma is rare. It affects 1 person per 200,000 each year in the U.S., according to the National Cancer Institute.
What are the stages of chondrosarcoma?
The most common type of chondrosarcoma is called conventional chondrosarcoma. Doctors give these tumors a grade based on how fast they're likely to grow:
- Grade 1 — Low-grade chondrosarcomas (or atypical cartilaginous tumors) grow slowly and are unlikely to spread.
- Grade 2 — Intermediate-grade chondrosarcomas are somewhat more likely to spread. (Most chondrosarcomas are grade 1 or 2.)
- Grade 3 — High-grade chondrosarcomas are most likely to spread.
What are the subtypes of chondrosarcoma?
There are also several chondrosarcoma subtypes, which make up about 10% of all chondrosarcoma cases in the U.S. every year. These tumors have unusual features that doctors can see under a microscope.
They include:
- Clear cell chondrosarcoma — These rare tumors grow very slowly and seldom spread to other parts of the body.
- Dedifferentiated chondrosarcoma — These start as typical chondrosarcoma, then the cells change to look and behave more like other types of bone cancer, such as osteosarcoma.
- Extraskeletal chondrosarcoma — This extremely rare form of chondrosarcoma forms in the soft tissues of the arms and legs.
- Mesenchymal chondrosarcoma — These aggressive tumors usually develop in younger adults. They may grow quickly and come back, even after treatment.
What causes chondrosarcoma?
Doctors and researchers aren’t sure what causes chondrosarcomas. The tumors may be linked to certain genetic changes in your chromosomes. It may also be linked to radiation exposure, injury, or genetic disorders. There are no environmental or lifestyle factors that cause chondrosarcoma.
Chondrosarcoma risk factors
Doctors aren’t sure why, but having certain health issues may make you more likely to develop chondrosarcoma.
You may be at greater risk for developing chondrosarcoma if you already have an underlying bone disease or abnormality. The following conditions are risk factors for chondrosarcoma:
- Family history — Your risk may increase if you have a family member who has been diagnosed with this type of tumor.
- Maffucci syndrome — A rare genetic disorder that causes benign (noncancerous) tumors in the cartilage of your hands. It can also cause abnormal tangles of blood vessels on your skin.
- Multiple hereditary exostoses — A genetic condition that causes benign tumors to form on bones. They typically form on the flat surfaces of bones or the cartilage at the ends of bones (also called the growth plates).
- Ollier’s disease — A condition where benign tumors in the bones cause those bones to swell. It may be present at birth but may not be apparent until early childhood.
- Paget’s disease — A chronic disorder where your body makes too much new bone, resulting in misshapen bones that are larger and weaker than normal.
Complications of chondrosarcoma
Complications of chondrosarcoma include increasing pain or a growing lump where the tumor is. You may have a hard time moving the affected limb. Conventional chondrosarcomas grow slowly, but some aggressive forms can metastasize (spread) to other organs of the body.
If the tumor is pressing on the spine, you may experience memory problems or issues with bladder or bowel control. If the tumor metastasizes into the lungs, you may have trouble breathing. The cancer can eventually lead to death.
How can I prevent chondrosarcoma?
Because there are no known environmental or lifestyle factors that cause chondrosarcoma, there's no way to prevent it.
What Are the Signs and Symptoms of Chondrosarcoma?
Many people with chondrosarcoma don’t have any signs of the disease at first. The main symptom is bone pain that may come and go or get worse at night.
Other symptoms may include:
- A fracture after a minor fall or injury.
- A limp or restricted movement of a joint.
- A lump on your bone that you can feel.
- Fatigue.
- Numbness or tingling (if the tumor is pressing on the nerves of the spine).
- Pain that gets worse at night and doesn’t go away with rest.
- Pain with weight bearing or other activities.
- Swelling around the tumor area.
- Unexplained weight loss.
Symptoms of chondrosarcoma of the skull base (petroclival synchondrosis) may include:
- Double vision.
- Headache.
- Tinnitus — Ringing in the ears.
When should I see a doctor about my chondrosarcoma symptoms?
If you have any symptoms of chondrosarcoma, such as bone pain, or feel a lump, see a doctor as soon as possible. Early treatment reduces the risk that the cancer will spread and improves your chance of a positive treatment outcome and overall survival.
Because chondrosarcomas are so rare, your primary care provider (PCP) may not have seen this condition before. They may refer you to an orthopaedic specialist for diagnosis if they suspect bone cancer.
How Do You Diagnose Chondrosarcoma?
To diagnose chondrosarcoma, your doctor will ask you about your symptoms and medical history and perform a physical exam. Because chondrosarcoma is a rare disease, your care team may include orthopaedic surgeons and oncologists with specialized knowledge of bone cancer.
What to expect during your visit
If your doctor suspects chondrosarcoma, they will:
- Ask you about your medical history and your family’s medical history.
- Ask you about your symptoms.
- Perform a physical exam, focusing on the areas causing pain.
- Order imaging tests.
Tests to diagnose chondrosarcoma
Imaging studies and procedures help your doctor diagnose and locate the chondrosarcoma, and may include the following tests in this order:
- X-ray — A diagnostic test that uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs. X-rays are often the first step in imaging and workup for bone pain or bone tumors.
- CT scan — A test that creates images of your body's organs, bones, and tissues.
- MRI — Uses a combination of large magnets, radio frequencies, and a computer to produce detailed images that pinpoint the tumor, helping doctors develop a treatment plan.
- Biopsy — A procedure that takes a small sample of tumor tissue for analysis in a lab. A biopsy can determine if a tumor is cancerous.
Your doctor may also order blood tests, bone scans, or PET scans to gain more information.
Chondrosarcoma prognosis
Your prognosis after a chondrosarcoma diagnosis depends on the size, location, and extent of your tumor, and whether it has spread to other areas of your body. Your doctor will discuss your prognosis with you.
What is the survival rate for chondrosarcoma?
The survival rate after a chondrosarcoma diagnosis depends on where the tumor is located and whether it has spread to other areas of your body. Approximately 79% of people survive for at least five years after a chondrosarcoma diagnosis.
How Do You Treat Chondrosarcoma?
The goals of chondrosarcoma treatment are to remove the tumor, prevent the cancer from spreading to other parts of your body, eliminate symptoms, and reduce your risk of complications. Surgical removal is the preferred treatment for most chondrosarcomas. Doctors may use other therapies as well, depending on the type, grade, and location of your tumor.
Treatment options may include:
Surgery for chondrosarcoma
Surgery is the standard procedure for most conventional chondrosarcomas.
Doctors may use one of the following surgical techniques:
- Curettage — After scraping the tumor cells out of the bone, doctors may fill the empty space with bone cement. Doctors usually use this technique on tumors that are less likely to spread.
- Wide resection — This involves cutting out the tumor and a section of healthy tissue near the tumor. If the cancer is in an arm or leg, surgeons may need to remove the entire limb.
- Reconstruction — Whenever possible, surgeons will preserve as much healthy tissue as possible and reconstruct the arm or leg (limb-salvage surgery). For that, they may need a bone graft of donated bone or bone taken from elsewhere in your own body. You may need a metal implant in the bone or an artificial joint.
Minimally invasive surgery to treat chondrosarcoma of the skull base
At UPMC, the preferred surgical treatment for chondrosarcoma of the skull base is the endoscopic endonasal approach (EEA). This innovative, minimally invasive technique uses the nose and nasal cavities as natural corridors to access hard-to-reach or previously inoperable tumors, without the need for an incision.
Benefits of EEA include:
- Faster recovery time.
- No disfigurement.
- No incisions.
If you need additional treatments, such as radiation or chemotherapy, those therapies can begin soon after EEA surgery.
Chemotherapy and radiation therapy
Your doctor may recommend chemotherapy and radiation therapy to treat your chondrosarcoma. In general, these therapies are less commonly used in the treatment of chondrosarcoma, but your care team will help you understand if these techniques may be beneficial for your specific situation.
Chemotherapy
Chemotherapy medications for chondrosarcoma kill cancer cells and may be:
- Injected.
- Placed inside the body near the tumor site.
- Taken by mouth.
Radiation therapy
Radiation therapy uses x-ray energy to kill cancer cells. This treatment may be an option for tumors that can’t be removed with surgery. For instance, if the tumor is enmeshed in the spinal cord.
Radiation therapy treatment options include:
- External radiation — Directs radiation at the tumor from an outside source.
- Internal radiation — Involves placing radioactive material in the body near the tumor.
How effective is treatment?
How effective your treatment for chondrosarcoma is depends on:
- If doctors were able to remove all the tumors during surgery.
- The grade of the chondrosarcoma (how abnormal the cells are).
- The stage of the cancer at the time doctors discovered it.
- What type of chondrosarcoma you have. (Both dedifferentiated and mesenchymal chondrosarcomas grow much faster than conventional chondrosarcomas.)
- Where the tumor was in your body. (Some tumors, such as those in the skull, spine, or pelvis, are more difficult to treat and remove.)
- Whether the cancer was confined to one area or had spread to other parts of the body.
Why Choose UPMC for Chondrosarcoma Care?
When you choose UPMC for chondrosarcoma care, you will receive:
- Access to world-class surgical expertise — Our world-renowned experts treat all types of chondrosarcoma tumors using the latest approaches and techniques.
- A full range of treatment options — We perform minimally invasive and traditional open surgical procedures to effectively treat all types of tumors while reducing your risk of complications.
- Multidisciplinary care — We partner with skull base surgeons, neurologists, orthopaedic surgeons, radiology oncologists, medical oncologists, and other medical experts to provide complete care that optimizes your recovery and quality of life.
