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Chondrosarcoma

Chondrosarcoma is a group of rare bone tumors, 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.

The most common treatment for chondrosarcoma is surgery.

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What Is Chondrosarcoma? 

Doctors define chondrosarcoma as a type of primary bone cancer in which malignant tumors begin in the bone, as opposed to cancers that spread to the bone from another organ. Chondrosarcoma is the second most common type of primary bone cancer, after osteosarcoma.

Other types of primary bone cancer are:

  • Ewing’s sarcoma. This bone cancer occurs most often in older children and teens, typically in the upper and lower leg, pelvis, upper arm, or ribs.
  • Osteosarcoma. This aggressive bone cancer occurs in teenagers and young adults, usually in the thighbone or shinbone.

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.

Image of Osteosarcoma, Chondrosarcoma and Ewing Sarcoma.

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 small numbers of 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 aren’t sure what causes chondrosarcoma. The tumors may be linked to certain genetic changes in your chromosomes. There are no environmental or lifestyle factors that cause chondrosarcoma.

What are chondrosarcoma risk factors and complications?

Doctors aren’t sure why, but having certain health issues may make you more likely to develop chondrosarcoma.

Chondrosarcoma risk factors 

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:  

  • 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) into 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. 

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What Are the Signs and Symptoms of Chondrosarcoma?

Many people with chondrosarcoma don’t have any signs of the disease at first.

Others may have one or more of the following chondrosarcoma symptoms:

  • A fracture after a minor fall or injury.
  • A limp or restricted movement of a joint.
  • A mass that can be felt on the affected bone.
  • Numbness or tingling (if the tumor is pressing on nerves of the spine).
  • Pain that gets worse at night and doesn’t go away with rest.
  • Pain with activity.
  • Swelling around the tumor.

When should I see a doctor about my chondrosarcoma symptoms?

You should see a doctor if you have any of the symptoms above. Because chondrosarcomas are so rare, your primary care provider (PCP) may not have seen this condition before. They'll refer you to an orthopaedic specialist for diagnosis if they suspect bone cancer.

How Do You Diagnose Chondrosarcoma?

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

Your doctor will take your medical history and ask you questions about your overall health and any symptoms you’re experiencing. They'll ask if there's any history of cancer in your family or if you have any inherited conditions that could be risk factors for chondrosarcoma.

Your doctor will perform a physical exam, focusing on the areas causing pain.

Tests to diagnose chondrosarcoma

Your doctor will start by ordering x-rays to view images of your bones.

Then, they may order the following tests to diagnose chondrosarcoma:

  • A biopsy. Doctors will remove a small piece of tissue from the tumor and look at it under a microscope. They'll be able to confirm where the tumor started, and what grade it is.
  • Blood tests. These tests can determine how well the rest of your body is working.
  • More imaging tests, including bone, CT, and MRI scans, or PET scans. These tests will allow your doctor to see where the tumor is and how big it is. They can also see if there are signs that the tumor has spread to other parts of the body. 

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How Do You Treat Chondrosarcoma? 

The most common treatment for chondrosarcoma is surgery to remove the tumor. Doctors may use other therapies as well, depending on the type, grade, and location of your tumor. 

Surgery for chondrosarcoma

Surgery is 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. 

Medicine to treat chondrosarcoma

Chemotherapy, which doctors use to treat many cancers, isn't usually used to treat chondrosarcoma. That’s because chemotherapy targets rapidly dividing cells.

Since chondrosarcomas tend to grow slowly, they typically don’t respond well to chemotherapy. However, doctors may use chemotherapy on some tumors that are very aggressive or have spread to different parts of the body.

Other treatments for chondrosarcoma

Other potential treatments for chondrosarcoma include:

  • Cryosurgery, which uses very cold liquid to destroy the tumor.
  • Radiation treatments, which 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 may be the best choice.

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.

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