Chondrosarcoma in Children

Chondrosarcomas can be rapidly invasive or slow-growing and cause fewer symptoms and seldom spread to other organs and tissues. Several factors, including Ollier’s disease (multiple benign tumors within bone) and a rare genetic disorder called Maffucci syndrome, may contribute to disease risk.

Chondrosarcoma symptoms may include:

• Pain (sharp or dull) at the tumor site
• Swelling and/or redness at the tumor site
• Enlargement of pre-existing exotoses (bone growths)
• Increased pain with activity or lifting
• Limp
• Decreased movement of affect limb
• Urinary obstruction (with pelvic tumors)

A physician may order a number of different tests to diagnose chondrosarcoma. In addition to a medical history and physical exam, these may include:

• Physical exam and neurologic function tests including reflexes, muscle strength, eye and mouth movement, coordination and alertness
• Magnetic resonance imaging (MRI) to produce detailed images of organs and structures within the body and/or spine
• Magnetic resonance spectroscopy (MRS), which is a test done along with MRI at specialized facilities that can detect the presence of particular organic compounds produced by the body's metabolism within sample tissue
• Computerized tomography scan (also called a CT or CAT scan) to capture a detailed view of the body
• Biopsy or tissue sample from the tumor to provide definitive information about the type of tumor; this is collected during surgery
• Lumbar puncture (spinal tap) to remove a small sample of cerebrospinal fluid and determine if any tumor cells have started to spread. In young children, this procedure is safely performed under sedation, and is less difficult and less painful than placing an intravenous (IV) catheter.

Chondrosarcoma treatment for children may include:

• Surgery: Surgery is often a first step, allowing doctors to form a complete diagnosis of the tumor type and providing information on the stage of the disease. If your child’s leg or arm is affected, your child may receive:
• limb-salvage surgery, to help preserve the limb by removing the chondrosarcoma tumor and wide margins of healthy tissue surrounding the tumor
• rotationplasty, a partial amputation that preserves a cancer-free lower leg, attaches it to the thighbone and uses the ankle as a knee joint
• amputation surgery, which in rare cases may be necessary (for example, if it involves the nerves and blood vessels)
• Head or neck surgery, if the chondrosarcoma is in the head or neck; this involves surgical removal of the tumor and bone/skin grafts.

The type of surgery will depend on the size and location of the tumor, and whether the cancer has spread. At Dana-Farber/Boston Children's, these procedures are performed in the hospital's new state-of-the-art pediatric MR-OR. It allows specialists to take an MRI scan immediately following surgery to ensure the tumor is completely removed and decreases the need for additional surgery.

• Radiation therapy: This treatment can help stop the growth of abnormal cells in specific areas of the body. Radiation therapy uses high-energy rays from a specialized machine to damage or kill abnormal cells. Our doctors use precisely targeted and dosed radiation to kill cancer cells left behind after your child's surgery.

• Chemotherapy: These medicines can help stop the growth of abnormal cells throughout the body. Chemotherapy is systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to kill or slow the growth of targeted cells. Different groups of chemotherapy drugs work in different ways, and can be taken in a variety of ways including orally or intravenously.

Prognosis for children with chondrosarcoma greatly depends on:

• the extent of the disease
• the size and location of the tumor
• presence or absence of metastasis
• the tumor's response to therapy
• the age and overall health of your child
• your child's tolerance of specific medications, procedures or therapies
• new developments in treatment

Every child is unique and treatment and prognosis is structured around your child's needs. Prompt medical attention and aggressive therapy are important for the best prognosis.

Survivorship care

Children treated for chondrosarcomas should visit a cancer survivorship clinic every year to:

• manage disease complications
• screen for early recurrence of cancer
• manage late effects of treatment

A typical follow-up visit may include some or all of the following:

• a physical exam
• laboratory testing
• imaging scans

Through our David B. Perini, Jr. Quality of Life Clinic, childhood cancer survivors receive a comprehensive follow-up evaluation from their cancer care team. In addition to meeting with your pediatric oncologists, your child may see one of our endocrinologists, cardiologists, neurologists, neuro-psychologists or alternative/complementary therapy specialists.