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While chondrosarcomas are rare, with fewer than 2,000 new cases diagnosed in the U.S. each year, they are the most common primary bone cancer in adults. They usually occur in people over the age of 40 years and can be challenging to diagnose because cartilage cells are usually slow-growing.

Types of Chondrosarcomas

There are several types of chondrosarcomas.

Conventional (Primary) Chondrosarcoma. This is the most common type, accounting for the majority of chondrosarcoma diagnoses.

High-grade chondrosarcomas (grade II or grade III) are most common in men in the sixth to eighth decade of life. These cancerous lesions are generally located in the long bones or the pelvis. The most common locations are the pelvis, proximal femur (upper part of the femur that connects to the hip), ribs, humerus, tibia, and scapula (shoulder blade).

There are also some more aggressive sub-types:

• De-differentiated chondrosarcoma. De-differentiated chondrosarcoma is the most aggressive type of chondrosarcoma. It accounts for approximately 10% of chondrosarcomas. De-differentiation means that the cancerous cartilage cells de-differentiate (mutate) into other cancerous cells, like osteosarcoma or sarcoma cells.
• Mesenchymal Chondrosarcoma. Mesenchymal chondrosarcoma is rare (accounting for less than 1% of chondrosarcomas) and can develop in both bone and soft tissues. It is most frequently found in adults in their third decade of life. The most common locations are the jaw, pelvis, spine, and scapula (shoulder blade).
• Clear-Cell Chondrosarcoma. Clear-cell chondrosarcoma is another rare subtype, accounting for less than 5% of all chondrosarcomas. It is most commonly found in the end of the bone, usually at the ball of the hip in younger patients (20 to 40 years old). Some oncologists believe that clear-cell chondrosarcoma is the cancerous counterpart of chondroblastoma.

Secondary Chondrosarcomas. Some chondrosarcomas develop from benign (noncancerous) bone tumors, such as an enchondroma or an osteochondroma. These are known as secondary chondrosarcomas. However, this occurs in less than 1% of solitary enchondromas and osteochondromas.

Patients with secondary chondrosarcomas have a better prognosis (outlook) than patients with other chondrosarcomas. In general, these chondrosarcomas are low-grade, slow-growing lesions that have low potential to metastasize (spread beyond the original site).

For most cases of primary chondrosarcoma, there is no known cause or risk factor.

A small subset of patients have a higher risk of developing a secondary chondrosarcoma due to another disease:

• Multiple hereditary exostosis
• Ollier disease
• Maffucci syndrome

The signs and symptoms of cartilage lesions vary depending on the location and type of the lesion.

Malignant Lesions

Patients with malignant chondrosarcomas typically have normal bloodwork and other laboratory tests. Common symptoms include:

• A history of progressive pain (pain that gradually gets worse over time).
• Pain that is constant, not relieved with rest, and often worse at night (it often interferes with sleep).
• Sometimes, a mass that grows over time.
• Possibly, swelling of the affected limb.
• Lesions in the arms or legs can cause pain, weakness, and disability in the limb. Range of motion of the joint near the lesion may be limited, and there may be atrophy (wasting, or shrinking) of the limb.

Benign (Non-Cancerous) Lesions

Benign cartilage lesions (enchondromas) of the long bones are usually:

• Asymptomatic (do not cause symptoms).
• Not painful. When there is pain, it is typically caused by injuries to other structures near the lesion (e.g., tendons or muscles) or arthritis in the nearby joint. This is especially common in enchondromas of the proximal humerus (shoulder), in which the cause of the patient’s pain is rotator cuff damage, not the bone tumor.
• Found incidentally (by accident) when a person has an X-ray for an injury. In addition to showing the injury, the X-ray reveals a tumor.

Enchondromas involving the short tubular bones of the hand are often associated with pathologic fractures. Pathologic fractures are fractures that occur with minimal trauma because the bone is weakened by an underlying health condition (like osteoporosis or cancer). This is most commonly seen in women in the fourth to eighth decades of life.

The first step toward getting a proper diagnosis is to visit your doctor. If chondrosarcoma is suspected, it is recommended that you get a referral from your primary care or family medicine doctor to see an orthopaedic oncologist.

Medical History and Physical Examination

Your doctor will start by:

• Taking a detailed medical history to learn more about your symptoms. Be prepared to discuss your medical problems, surgical history, medications, and family cancer history.
• Asking questions about the nature and location of your pain, previous injuries to that specific area, changes in the appearance of the bone, whether you discovered a firm mass on the bone, and any factors that make the symptoms better or worse (e.g., the pain comes on during activity and goes away with rest).
• Performing a physical exam to better understand what is going on in your body.

Imaging Tests

Your doctor will then order imaging tests, typically starting with X-rays. Sometimes, they will also order magnetic resonance imaging (MRI) scans and/or computed tomography (CT) scans.

X-rays. Cartilage lesions can often be identified by their radiographic appearance (how they look on an X-ray).

On X-ray:

• Enchondromas have a classic appearance of white “popcorn” calcifications without any aggressive features that would change or damage the surrounding bone.
• Chondrosarcomas have a more aggressive radiographic appearance. The X-ray will show destruction of bone, a large soft tissue mass, and possibly a fracture.

Benign-appearing cartilage tumor most consistent with enchondroma.

Aggressive-appearing cartilage tumor with bony destruction and soft-tissue mass most consistent with chondrosarcoma.

Advanced Imaging

Additional imaging tests are often helpful in the diagnosis and staging of cancerous cartilage lesions.

Magnetic resonance imaging (MRI) scan. MRI is useful in determining how much of the tumor is within the bone cavity (medullary cavity). It is thought that when a larger percentage of the tumor is within the bone cavity, the tumor is more likely to be cancerous. MRI will also show the size, borders, and precise location of the soft tissue mass.

Computed tomography (CT) scan. CT scans can be used to further evaluate the structure of the mass and show the pattern and extent of the bone cancer.

Staging

After a diagnosis of chondrosarcoma is established, complete staging is recommended. Complete staging means having:

• A chest CT – The lungs are the most common location for chondrosarcoma to metastasize (spread to another location in the body).
• A whole-body positron emission tomography-computed tomography scan (PET CT scan), or bone scan. Both of these scans can be used to look for metastasis (spread of the cancer) to other areas of the body.

Biopsy

• Biopsy is not recommended for cartilage lesions where X-rays have a benign appearance and the patient does not have any symptoms (as with enchondromas or atypical cartilaginous tumors).
• Biopsy may be recommended for a suspected chondrosarcoma. It can be performed as an open biopsy (by a surgeon) or as a core-needle biopsy (performed by surgeon or interventional radiologist). Core-needle biopsy is an excellent option when the doctor wants to confirm the findings of X-rays and clinical examination. It is performed with the help of real-time imaging, such as fluoroscopy or CT. Occasionally, it can be difficult to establish the diagnosis of a cartilage tumor even with a biopsy. Sometimes, the final grade or subtype of the tumor cannot be determined until the tumor is resected (surgically removed).

The decision to perform a biopsy and the technique used should be made by an experienced orthopaedic oncologist who takes into account the patient's history, physical examination, and X-ray results.

Treatment of chondrosarcoma depends on many factors, including:

• The patient's age
• The patient's overall health
• The presence of other medical conditions
• The location of the tumor
• Whether the cancer has metastasized (spread)
• The specific sub-type of chondrosarcoma

Although treatment is individualized, most chondrosarcomas are treated with surgery alone.

The goal of surgery is a wide surgical resection to remove all of the cancer. This means your orthopaedic surgeon will remove all of the bone with cancer along with a margin of healthy tissue around the tumor to ensure they are able to clear all cancer cells from your body.

Due to the relatively slow growth of cartilage cells, chemotherapy or radiation therapy have not been found to be beneficial for most chondrosarcomas.

• Chemotherapy may be used to treat certain sub-types, such as mesenchymal and de-differentiated chondrosarcomas.
• Radiation therapy may be recommended when a tumor cannot be surgically removed or for metastatic disease. Radiation therapy should also be considered if there are positive margins at time of surgery, meaning microscopic cancers cells may have been left behind in the body when the tumor was removed.

Here is a summary of how each sub-type is typically treated.

Conventional, High-Grade Chondrosarcoma

Patients with multiple hereditary exostosis (MHE) or multiple enchondromas (Ollier's disease or Maffucci syndrome) have a higher risk of developing a secondary chondrosarcoma.

• About 10% of patients with MHE and 25% of patients with Ollier disease will develop a secondary chondrosarcoma.
• Patients with Maffucci syndrome have up to a 100% risk of developing a malignancy (chondrosarcoma or carcinoma) sometime in their life.

These patients should be followed over time with a series of X-rays.

Clear-Cell Chondrosarcoma

Clear-cell chondrosarcoma is treated with wide local excision (surgical removal).
The outlook in patients with clear-cell chondrosarcoma tends to be better than for patients with other chondrosarcomas. These lesions can metastasize to the lung late; therefore, long-term follow-up for patients with metastasis to the lungs is needed.

Mesenchymal Chondrosarcoma

This subtype of chondrosarcoma is typically treated with chemotherapy, in addition to surgery (resection, or removal, of the tumor). Occasionally, radiation may be recommended.

With mesenchymal chondrosarcoma, there is a high likelihood of metastases to the lung, lymph nodes (unlike conventional chondrosarcoma), and other bony structures.

De-differentiated Chondrosarcoma

The treatment regimen for de-differentiated chondrosarcoma is similar to treatment for high-grade osteosarcoma — chemotherapy and surgical resection.

Occasionally, radiation may be recommended.

Intermediate Lesions (Atypical Cartilaginous Tumors)

An atypical cartilaginous tumor (ACT) was previously classified as a low-grade chondrosarcoma (grade I chondrosarcoma).

However, after decades of studying patients with low-grade chondrosarcomas in the arms and legs, physicians recognized that these tumors never required more than a small surgery and did not spread to other sites in the body (no metastasis). Accordingly, these tumors were re-classified as ACTs.

An ACT tumor can be treated with a curettage (scraping the tumor out), with no need for added treatments.

The main goal of tumor surgery is to remove the tumor entirely and keep it from returning. The secondary goal is to reconstruct the limb to offer the patient the best possible function. Most of the time, limb salvage surgery is possible, though occasionally, an amputation is the best option.

Sarcoma surgery requires surgeons to remove all of the bone affected by tumor and a margin (border) of healthy tissue around the tumor. Then, orthopaedic surgeons can reconstruct the bone, joint, or soft tissue site.

Most commonly, there are several options for reconstruction that may use bone grafts, artificial joints, or a combination of both. The goal is to restore the body part so that the patient can participate in their normal everyday activities.

You can think of reconstruction options using the following categories:

• Arthroplasty — large joint replacements with metal parts replacing damaged natural parts
• Allograft — using cadaver bone to rebuild the bone that was removed
• Autograft — using the patient's own bone, such as a fibula, to reconstruct the bone removed
• Allograft prosthetic composite (APC) — a combination of cadaver bone and joint replacement
• Amputation (including rotationplasty)

How much function can be restored from reconstructive surgery depends on how much muscle and joint tissue can be preserved (saved) while still removing the entire tumor. The surgeon’s primary goal is always to help the patient become cancer-free and lower the chance that the tumor will come back.

Recovery after limb salvage surgery requires a lot of physical therapy, rehabilitation, and follow-up care. Patients need to be encouraged and supported by family and friends during this time. It can be challenging to participate in rehabilitation due to reduced energy level and lack motivation to exercise after a major surgery.

The rehabilitation plan will vary based on the type of surgery that was performed.

Most patients notice improvement in strength, function, and mobility within 1 to 2 years after surgery; after 2 years, there will not be much, if any, additional improvement.

Surveillance

Regular follow-up visits will be needed after treatment for chondrosarcoma to monitor your overall condition, and check for complications from treatment and cancer recurrence or spread.

These visits will include regular imaging (such as X-rays, CT scans, and/or MRIs) and physical exams. Your doctor may also order blood tests.

The patient's outlook after treatment will depend on many factors, including:

• Age
• Overall health
• The presence of other medical conditions
• The stage and grade of the tumor
• Whether the cancer has metastasized (spread to other locations)
• The specific sub-type of osteosarcoma
• Response to chemotherapy

In most cases, the grade of the tumor is the best predictor of how the patient will do after treatment. At the time the tumor is removed, the pathologist will examine it under a microscope to determine the final grade and sub-type. In general, the lower the grade, the better the outlook.

Treatment is successful for many patients with chondrosarcoma. However, because each situation is unique, it is best to talk to your treatment team about the specific prognosis for your cancer.