Overview of Chondrosarcoma

Chondrosarcomas are rare bone cancers that most commonly occur in the bones of the arms and legs, shoulder blade, and pelvis. Neurosurgeons treat patients with chondrosarcomas in the spine or the skull. Chondrosarcomas account for less than 1% of all bone cancers, with 2 to 4 cases for every 1 million people per year.

Chondrosarcoma develops from the cartilage cells within bones. These cells can become cancerous because of genetic mutations and divide uncontrollably to form a tumor. Although chondrosarcomas usually occur in people older than 40 years, they can occur in younger individuals too. People who have been exposed to excessive amounts of ionizing radiation are at a higher risk of developing tumors, including chondrosarcoma.

If more than one person in a family is diagnosed with chondrosarcoma or other bone tumors, there might be a genetic link. Certain genes like EXT1, EXT2, IDH1, IDH2, and INK4A have been found to be associated with a higher chondrosarcoma risk. In addition, genetic conditions like Ollier disease, Maffucci syndrome, and multiple hereditary exostosis can increase the risk for developing chondrosarcoma. However, most people with chondrosarcoma do not have a known genetic link, and many patients do not have any family members with bone tumors.

Most people with chondrosarcoma do not have any known risk factors. Even if you or another family member has a genetic risk, this does not mean that you for certain will develop chondrosarcoma. At present, little is known on how to prevent chondrosarcoma, but having it diagnosed and treated early helps improve prognosis. For this reason, it is a good idea to have regular checkups with your primary physician to evaluate any unusual growths or symptoms.

Chondrosarcomas can cause a variety of symptoms based on the location and size of the tumor. Chondrosarcoma of the skull base can cause:

• Headache
• Visual disturbances
• Difficulty with balance
• Dizziness
• Hearing difficulty
• Facial pain or numbness
• Facial asymmetry
• Difficulty swallowing

People with chondrosarcomas of the spine may experience:

• Back pain
• Change in bowel and bladder habits
• Numbness in the legs
• Difficulty walking because of weakness in the legs

These symptoms may be indicative of various conditions, so it's crucial to consult a doctor for an accurate diagnosis. Don't hesitate to seek medical advice if you are worried about any symptoms. When discussing your medical history, make sure to inform your doctor about any family members with a cancer diagnosis.

Your physician might suggest imaging tests like magnetic resonance imaging (MRI) or a computed tomography (CT) scan to identify and analyze any tumors, offering information on their location, size, and impact on surrounding structures. Armed with this information, you and your doctor can collaboratively determine the most appropriate course of action.

Diagnosis of chondrosarcomas affecting the brain and spine typically involves a combination of medical evaluation, imaging scans, and biopsy. If you are being evaluated for a possible chondrosarcoma, the following diagnostic procedures may be ordered:

• Medical history and physical examination: Your doctor will inquire about your symptoms and medical background and perform a physical examination to assess any indications of a tumor or abnormal growth.
• Imaging tests: Imaging tests such as X-rays, CT scans, MRI, or positron emission tomography (PET) scans may be used to assist your doctor in identifying a tumor, determining its size, location, and shape.
• Biopsy: A biopsy involves the removal of a small tissue sample from the tumor, which is then examined under a microscope. This procedure helps to confirm a diagnosis of chondrosarcoma and determine the tumor’s grade.
• Additional tests: Additional tests like blood tests, bone scans, and other imaging exams might be conducted to eliminate other conditions or evaluate the extent of the cancer.

Detecting chondrosarcoma in the brain and spine is challenging and necessitates a multidisciplinary approach involving a team of specialists including a neurologist, neurosurgeon, oncologist, radiologist, and pathologist. This group of experts collaborates to review test results, confirm the diagnosis, and develop an appropriate treatment plan.

When doctors confirm a case of chondrosarcoma, they use a tissue biopsy to determine its subtype, grade, and stage. Doctors group chondrosarcomas into different types based on their appearance under a microscope, including subtypes such as classical or conventional, dedifferentiated, mesenchymal, and clear cell types.

The classical or conventional subtype is the most frequently seen form of chondrosarcoma. Dedifferentiated and mesenchymal subtypes are more aggressive, meaning they grow quickly and are likely to spread, and generally have a less favorable prognosis.

The grade of a chondrosarcoma tells us how abnormal the cancer cells are, and the stage tells us how much the cancer has spread in the body. If the grade or stage is higher, it usually means the outlook or prognosis is poorer.

There are 3 grades of chondrosarcoma:

• Low-grade (Grade 1) chondrosarcoma: These tumors grow slowly and are not likely to spread to other parts of the body.
• Intermediate-grade (Grade 2) chondrosarcoma: These tumors grow faster than low-grade ones and have a higher chance of spreading.
• High-grade (Grade 3) chondrosarcoma: These tumors grow quickly and are more likely to spread to other parts of the body.

Chondrosarcoma can also be described by its stage, which reflects how big the tumor is and if it has spread to other parts of the body. Staging chondrosarcoma can be complicated and depends on where the tumor is and its specific characteristics. A team of specialists will work together to determine the stage of your tumor and come up with the best treatment plan.

The stages of chondrosarcoma are determined using a system called the American Joint Committee on Cancer (AJCC) staging system. The stages are:

• Stage 0: The tumor is only in the cartilage.
• Stage I: The tumor is in the bone and is smaller than 8 centimeters (about 3 inches).
• Stage II: The tumor is in the bone and is larger than 8 centimeters (about 3 inches).
• Stage III: The tumor has spread to nearby soft tissues, like muscles or ligaments.
• Stage IV: The tumor has spread to other parts of the body, like the lungs or other bones.

Chondrosarcoma is a kind of bone cancer that can spread to other parts of the body. Cancer cells from the main tumor in the bone can travel to other places through blood or lymph vessels. This spread can happen early on or many years later.

High-grade chondrosarcomas often have metastasized or spread. Lungs are a common place for metastasis. At first you might not notice any symptoms if the cancer has spread to the lungs. However, as the cancer gets worse, you may experience trouble breathing, bloody coughs, fatigue, loss of appetite, sudden weight loss, or feel sick to your stomach.

Not all patients have symptoms when the cancer spreads. Sometimes a metastasis is found during a regular test to check the main tumor. If you think you might have chondrosarcoma or have been diagnosed, make sure to follow your doctor’s recommendations for testing, checkups, and screening evaluations so that any tumor can be monitored and treated.

If you have any concerns about symptoms or a possible chondrosarcoma, consult a knowledgeable specialist to help confirm your diagnosis and form a treatment plan.

• Chondrosarcoma is a rare bone cancer that develops from the cartilage cells in bones.
• It most commonly occurs in the bones of the arms, legs, pelvis, spine, and skull.
• Chondrosarcoma can cause a variety of symptoms based on the tumor size and location.
• Diagnosing a chondrosarcoma requires a detailed physical examination by a doctor, MRI and CT scans, and a biopsy.
• The higher the grade or stage of the chondrosarcoma, the less favorable the outlook and prognosis.

• Sarcoma Foundation of America
• The Liddy Shriver Sarcoma Initiative
• Chondrosarcoma Foundation
• National Brain Tumor Society
• American Brain Tumor Association