Chondrosarcoma

What is chondrosarcoma?

Chondrosarcoma is a type of malignant tumor that mimics the cartilage that coats the ends of bones where a joint is formed. Cartilage is the flexible connective tissue found in the joints between bones — around the knee for example — and also found in the rib cage, elbow, knee, ankle, bronchial tubes and spinal discs. It is not as hard as bone, instead it serves as a flexible "glue" to join one bone to another.

Chondrosarcoma occurs mostly in adults. It is extremely rare in young children and adolescents (ages 13-19). Chondrosarcoma is most often found in the upper leg bone, upper arm bone, shoulder blade, ribs and pelvis. The malignant cancer cells can be found inside a bone or on the surface of a bone.

Most chondrosarcomas are slow to grow and spread.

Causes

Chondrosarcoma occurs when abnormal cartilage cells divide and grow. Three conditions have been connected to a higher risk of developing chondrosarcoma:

  • Ollier disease, when a person has many enchondromas — benign (noncancerous) tumors made up of cartilage inside of bones
  • Maffucci syndrome, a disease where the person forms many enchondromas sporadically and hemagiomas (blood-filled birth-marks)
  • Multiple hereditary exostoses, where tumors with a cartilage cap grow on the surface of multiple bones

In most cases, these related conditions do not occur until adulthood.

Signs and symptoms

Symptoms of a chondrosarcoma depend on a variety of factors including the type of tumor, location of the tumor, and your child’s age and general health.

Indications of a chondrosarcoma may include:

  • Pain, stiffness or tenderness at the site of the tumor
  • Swelling or mass around the affected bone
  • Enlargement of a known osteochondroma (bone growth)
  • Limping or limited movement in the affected limb

Testing and diagnosis

Symptoms of a malignant musculoskeletal tumor can be difficult to identify in children, so prompt evaluation of any lump or mass is important.

Diagnosing a chondrosarcoma begins with a complete medical history and physical examination of your child. A detailed neurological assessment may also be performed. This involves a series of questions and tests to check the brain, spinal cord and nerve function.

At Children’s Hospital of Philadelphia (CHOP), clinical experts may use a variety of diagnostic tests to diagnose chondrosarcoma, including:

  • X-rays, which produce images of bones.
  • Magnetic resonance imaging (MRI), which uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of organs, soft tissues, muscles, ligaments and other structures within the body. Your child is exposed to no radiation during an MRI.
  • Computed tomography (CT) scan, which uses a combination of X-rays and computer technology to examine bones and produces cross-sectional images ("slices") of the body.
  • Needle biopsy, which is a procedure where a doctor places a small needle through the skin and into the lesion to withdraw a small sample of the abnormal tissue. The tissue is analyzed to confirm any findings.
  • Radioisotope bone scan, which can help locate areas of abnormal growth.
  • EOS imaging, an imaging technology that creates 3-dimensional models from two flat images. Unlike a CT scan, EOS images are taken while the child is in an upright or standing position, enabling improved diagnosis due to weight-bearing positioning.

Treatment

At CHOP, experts at the Bone and Soft Tissue Tumor Program take a team approach to treatment. Orthopaedic, cancer and other specialists collaborate to provide your child with individualized care and the best possible outcomes.

Our program is led by Kristy L. Weber, MD, and Alexandre Arkader, MD, nationally renowned surgeons who specialize in treating bone and soft tissues tumors, limb-sparing surgery and reconstructive surgery.

Unlike benign tumors that may only require “watchful waiting” to see if pain or dysfunction develops, malignant tumors such as chondrosarcoma require active treatment. For chondrosarcoma, surgical removal is always recommended.

Surgery

Surgery for chondrosarcoma has two goals:

  • To remove the tumor
  • To restore function at the site of the tumor

About 90 percent of children with chondrosarcoma can be treated with limb-sparing (also known as limb-salvage) and reconstructive surgery. CHOP surgeons regularly perform these complex surgeries and are constantly working to improve outcomes for children with the most difficult-to-treat tumors.

Limb-sparing surgery is performed under general anesthesia. It involves cutting out the chondrosarcoma and a margin of health tissue surrounding it. Depending on the size and location of the tumor, as well as your child’s age and stage of growth, surgeons may use a variety of reconstructive methods to restore your child’s body function.

Reconstruction may include:

  • Joint replacement if your child’s chondrosarcoma is located on or near her knee, hip or shoulder. If your child is still growing, a modified joint replacement that can be expanded as your child grows, may be used.
  • Allograft (cadaver bone) if your child’s tumor is in the middle of an arm or leg bone. This replacement bone serves as a structural filler — giving your child’s limb more strength and durability as his own bone grows around it.
  • Free vascularized fibular grafting which involves moving one bone from your child’s lower leg to reconstruct a defect where the disease bone was located. This may be done alone, or in conjunction with an allograft.

Depending on the surgery location and extent of any reconstruction, your child should expect to stay two to five days in the Hospital.

Surgical safety

Though surgery for chondrosarcoma is highly effective, we understand that any surgery can be a stressful experience for children and families. At CHOP, we offer a wealth of resources about how to prepare your child for surgery and what to expect during surgery.

Additionally, we employ numerous best practices before, during and after surgery to decrease the risk of infection and increase positive outcomes. For more details about safety protocols at Children's Hospital of Philadelphia, see safety in surgery.

Chemotherapy

Chemotherapy refers to medicines that help fight cancer. It is not effective at treating chondrosarcoma and therefore not used at CHOP. 

Radiation therapy

Radiation therapy uses high-energy waves such as X-rays to kill or shrink cancer cells. Chondrosarcoma tumors that cannot be removed surgically require high-dose radiation that can often only be delivered safely at a facility with proton therapy.

Follow-up care

After surgery, your child may require pain medication until the surgical site heals and ongoing physical therapy. Most children are encouraged to resume as active a life as possible.

Your child will see the orthopaedic surgeon about one to two weeks after surgery, then again every three months for two years post-surgery. Annual monitoring by trained clinicians is strongly encouraged for the next 20 years due to the slow-growing nature of chondrosarcoma.

Malignant tumors can reoccur — even after being successfully treated — so it is important for your child to see a physician regularly, especially if any symptoms recur.

During follow-up visits, X-rays and other diagnostic testing of the tumor site are recommended to closely monitor your child’s health.

Follow-up care and ongoing support and services are available at our Main Campus and throughout our CHOP Care Network. Our team is committed to partnering with parents and referring physicians to provide the most current, comprehensive and specialized care possible for your child.

If your child needs continued monitoring into adulthood, he can continue to see some of the same doctors who treated him. CHOP’s Bone and Soft Tissue Tumor Program works closely with Penn Medicine. For families who live farther away and need continued monitoring, our clinical professionals will help your child transition to adult care near home.

Outlook 

Children's Hospital of Philadelphia has an excellent track record of improving the quality of life for children diagnosed with chondrosarcoma.

Long-term outcomes for children with chondrosarcoma are varied and depend on the size, location and grade of the tumor (determined at the time of surgery); age and overall health of your child; and how your child responded to treatment.

With ongoing research and advances in treatment options, outcomes for young patients with chondrosarcoma will continue to improve.

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