Understanding chondrosarcoma: symptoms, treatment and prognosis

Chondrosarcoma is a rare type of bone cancer that develops in cartilage cells. It is the most common bone cancer in adults.

Conventional chondrosarcoma is the most common type of chondrosarcoma. These tumors are often located in the pelvis and the top part of the thigh and arm.

To learn more about chondrosarcoma, including symptoms to look out for, treatment options and prognosis, we tapped the experts: sarcoma medical oncologist Anthony Conley, M.D., and orthopedic oncology surgeon Justin Bird, M.D.

What are the different types of chondrosarcoma?

Conventional chondrosarcoma makes up about 80% of cases and usually occurs in people in their 50s and 60s, says Conley. These tumors tend to be slow-growing.

Other, rarer types of chondrosarcoma are:

• dedifferentiated chondrosarcoma
• mesenchymal chondrosarcoma
• clear cell chondrosarcoma

These types can grow quickly and spread. This often makes them harder to treat.

What causes chondrosarcoma?

It’s not known what exactly causes chondrosarcoma.

“We think sometimes cells in the bone get reprogrammed for reasons that are not clearly understood,” says Conley.

Some conditions can increase your chance of developing chondrosarcoma. These include:

Enchondromatosis

This is a rare disorder in which benign growths called enchondromas form in the bone. Sometimes, these growths can transform into chondrosarcoma.

Ollier disease

This is when people develop enchondromas all over the body, usually in the hands and feet. About 10% or fewer cases transform into chondrosarcoma.

Maffuci syndrome

This happens when people develop enchondromas as well as benign growths in their blood vessels, causing them to become enlarged.

Osteochondromatosis

This is a condition in which abnormal bone growths occur on the ends of long bones in the arms or legs. It can sometimes lead to chondrosarcoma. Osteochondromatosis can be hereditary, meaning it can be passed down from a parent to a child.

Primary synovial chondromatosis

This is when benign growths form in the synovium, the soft connective tissue that lines joints like the knee and elbow. Most can be removed with surgery, but sometimes a person will have one that degenerates and becomes a chondrosarcoma.

What are the symptoms of chondrosarcoma?

The most common symptoms of chondrosarcoma are:

• a lump
• pain or swelling near the tumor
• change in function, such as the ability to move your arm or leg correctly

“It’s easy for people to tolerate pain to a certain extent, but you should see a doctor if you feel a lump on your body that’s new and/or growing quickly,” says Conley. “Some doctors may assume it’s benign, like a lipoma. But if it continues to grow, cause pain or change in any way, go back to your doctor or seek a second opinion. At the end of the day, you are your best advocate.”

How is chondrosarcoma diagnosed?

If you have symptoms of chondrosarcoma, your doctor may order diagnostic tests. These could include:

X-ray

If you have symptoms related to the bone, an X-ray can provide information about the edges of the bone, the center of bone marrow and the bone’s overall architecture. If there is bone loss or bone growth where there shouldn’t be, that’s the trigger to get imaging done.

MRI or CT scan

An MRI is preferred, but you may get a CT scan if you have a medical device in your body that keeps you from getting an MRI. If doctors find a suspicious lesion during the scan, a biopsy is the next step.

Biopsy

During the biopsy, a piece of tissue is taken to be reviewed by a pathologist. The pathology results will determine if you have chondrosarcoma.

“After a diagnosis is made, the next step is staging the disease,” says Conley. “Is it localized? Are we dealing with one spot, or has it moved to other parts of the body? That’s when it becomes necessary to get imaging of the chest, preferably the abdomen and pelvis because chondrosarcomas can move to other organs.”

How is chondrosarcoma treated?

Treatment for chondrosarcoma depends on several factors, including the disease stage and grade, and whether the tumor can be removed surgically. Your care team will work together to find the best treatment plan for you. 

Surgery

Surgery is the most common treatment for chondrosarcoma.

“Some conventional chondrosarcomas can be cured with surgery,” says Conley.

Bird, who specializes in spine and pelvic tumors, often uses 3D modeling to help design the surgeries.

“We’re dealing with complex anatomy,” says Bird. “The models provide a physical representation of the tumor that is easier to process than MRI and CT scans because you don’t have to scroll through all the different imaging slices to get a sense of the entire tumor and relevant anatomy.” 

3D printing also assists with stereotactic navigation during surgery by allowing for more precision and accuracy.

“Surgery may be a good option for someone with a primary tumor, or single site of disease,” says Bird. “If you have a low-grade tumor in the finger, for example, we may surgically scrape it out. This is called an intralesional procedure where we remove the lesion piece by piece.”

But this type of surgery isn’t ideal for higher-grade tumors.

“We surgically remove high-grade tumors by taking it out all in one piece as well as the tissue around it to achieve negative margins,” says Bird. “This helps lower the chance for recurrence.”

Surgical treatment can also be more aggressive like amputation to remove a limb or a weight-bearing bone. These patients may receive physical therapy or occupational therapy after surgery.

Surgery is typically not an option for those with metastatic disease. At this point, the cancer cannot be cured.

“We’re researching to find new ways to treat metastatic chondrosarcoma that can’t be removed with surgery,” says Conley. “We’ll talk with the patient to see if they’d be a good fit for a clinical trial.”

Chemotherapy

More aggressive types of chondrosarcoma, like dedifferentiated chondrosarcoma and mesenchymal chondrosarcoma, often respond well to chemotherapy.

Patients with these types of diseases are given chemotherapy before surgery. They receive more chemotherapy after surgery to kill any residual microscopic disease. 

Radiation therapy

Sometimes, patients with localized disease, or cancer that is just in one area, are unable to get surgery for medical reasons. In those cases, radiation therapy may be used to treat the tumor.

What is the prognosis for people with chondrosarcoma?

How chondrosarcoma will affect you long-term depends on many factors, including the:

• location of the disease
• type of surgery you have
• technical skill of your doctors

Each patient is unique, so it’s important to discuss your prognosis with your doctor.

“It’s more common to see patients with localized disease, which can be stage I or stage II,” says Conley. “Overall survival for these patients can exceed 80% at five years, depending on the situation. And if you have stage I chondrosarcoma, your chance of surviving five years is going to be closer to 90% to 95%.”

Recurrent chondrosarcoma has a different prognosis

Prognosis becomes a bit trickier for patients who develop recurrent disease.

“The higher the grade, the higher the chance the chondrosarcoma will recur,” says Bird. “Low-grade tumors are less likely to recur, and when they do, they often grow back as low-grade. But sometimes things can change, and over time a low-grade tumor can transform and come back more aggressive.”

Not all stage IV disease is the same.

“Some people can relapse with 50 lumps, and some may relapse with only one lump. But, if it’s in a different organ, it’s classified as stage IV,” explains Conley. “The most common site of metastasis is the lung. If I have a relapsed patient with one or two spots on their lung, I believe we can potentially cure them. For relapsed patients with more advanced disease, curing the disease may not be possible. In those cases, our goal is to keep patients alive as long as possible with a preserved quality of life.”

TP53 mutation can affect chondrosarcoma prognosis

In recent years, genetic testing has helped to advance chondrosarcoma treatment.

For patients with recurrent chondrosarcoma, surgery and systemic therapies are not an option. In these cases, Conley says, MD Anderson will analyze the patient’s tumor tissue. What they find from the tissue can help them select targeted trials best suited for the patient.

An MD Anderson study published in 2023 found that patients with conventional and dedifferentiated chondrosarcoma who have the TP53 gene mutation have a poorer prognosis.

“For years, the only prognostic factor was the grade of the tumor,” says Conley. “But what we’ve proven through research is that the TP53 mutation can help determine prognosis as well. This helps us counsel patients during treatment.”

What should people keep in mind when deciding where to seek chondrosarcoma treatment?

If you are diagnosed with chondrosarcoma, it’s important to seek care at a comprehensive cancer center like MD Anderson with experts who have seen this type of cancer multiple times.

“At MD Anderson, there’s a multidisciplinary team of experts who deal with bone tumors,” says Conley. “They say it takes a village to raise a person. Similarly, it takes a village to treat chondrosarcoma. Each year, we see more than 200 bone tumor patients, including people with chondrosarcoma. When you see this disease as often as we do, you start to pick up patterns. We’re always researching new ways to treat chondrosarcoma and building upon that research to help more patients and their families.”

Request an appointment at MD Anderson online or call 1-877-632-6789.