What Is Chondrosarcoma? What Are Its Symptoms? How Is It Diagnosed and Treated?

Chondrosarcoma, a rare type of cancer that develops in the bones, originates from cartilage-forming cells. This disease can occur in any age group, but it is usually more common in people between 40 and 70 years of age. It is also seen more often in men than in women.

Among the symptoms of chondrosarcoma, bone pain, swelling and stiffness are the most common complaints. This type of cancer can occur in various forms. Among these, classic chondrosarcoma is the most common and accounts for approximately 85% of all cases. In this article, starting with the question of what chondrosarcoma cancer is, we will cover many important topics, including its types, diagnostic methods and treatment options for those undergoing chondrosarcoma surgery.

What is chondrosarcoma cancer?

Chondrosarcoma is a malignant bone tumor that originates from cartilage cells. This type of cancer develops from chondrocytes, which produce cartilage tissue, and causes abnormal production of cartilage tissue. Chondrosarcomas arise in bone tissue, but in some rare cases they can also develop within soft tissue.

Chondrosarcoma has an important place among primary bone cancers. In some countries, because of the increase in incidentally diagnosed cases, it is now regarded as the most common primary bone cancer.

These tumors can develop in two ways: as primary tumors (directly) or as secondary tumors (arising on the basis of pre-existing benign cartilage tumors such as enchondroma and osteochondroma). There are also different subtypes, and the most common subtype is classic chondrosarcoma.

In which age groups is it more common?

Chondrosarcoma usually occurs in adults between 40 and 70 years of age. The vast majority of cases are over 50 years of age. In the United States, the average age at diagnosis has been reported as 51, and more than 70% of patients are over 40 at the time of diagnosis.

However, while this disease is uncommon before the age of 30, it is quite rare before the age of 20 and in childhood. In particular, a rare subtype called mesenchymal chondrosarcoma is seen more often in the second and third decades of life (between 20 and 30 years of age).

Looking at the distribution by sex, chondrosarcoma is seen more often in men than in women. The male-to-female ratio is approximately 1.4 to 1.

Bone regions where chondrosarcoma is commonly seen

Chondrosarcoma can occur in bones in different parts of the body, but it appears more often in some regions. The most common sites are as follows:

• Pelvic region: Approximately 25% of all cases occur in the pelvic bones
• Long bones: Especially the proximal femur (hip), distal femur, proximal humerus (shoulder) and proximal tibia
• Rib bones
• Shoulder girdle

Interestingly, there are also differences in the distribution of bones in relation to the body axis. Appendicular chondrosarcoma (seen in the bones of the arms and legs) is more common in women, while axial chondrosarcoma (seen in the bones of the trunk) is more common in men.

The diagnosis and staging of chondrosarcoma can be difficult. For this reason, if symptoms such as bone pain and swelling appear, it is important to see a healthcare professional immediately.

What Are the Symptoms of Chondrosarcoma?

The symptoms of chondrosarcoma usually begin insidiously and may continue with mild complaints for a long time. In the early stages of the disease, symptoms may not be very noticeable, but as the tumor grows, various complaints begin to appear. The time between the onset of symptoms and diagnosis can sometimes take years.

Pain, swelling and stiffness

The most common symptom of chondrosarcoma is pain. This pain:

• Becomes more severe over time and is progressive
• Is usually dull in nature and does not go away with rest
• Gets worse especially at night and may disrupt sleep patterns
• May not respond as desired to painkillers

In addition to pain, you may feel a growing mass or swelling in the affected area [81]. This mass is usually hard and immobile, and tends to grow over time. Unlike benign cartilage lesions (enchondromas), the presence of pain in chondrosarcomas is an important clue in distinguishing benign from malignant.

Limited movement and limping

When chondrosarcoma develops near joints, it can cause limited movement. This may show itself with the following symptoms:

• Inability to move the affected joint fully
• Limping when walking in chondrosarcomas of the leg bones
• Reduced arm function in chondrosarcomas of the arm bones
• Over time, muscle wasting (atrophy) may be seen in the affected limb

As the tumor grows, it can press on surrounding tissues, and this may limit your mobility even further. In some patients, neurological symptoms such as numbness or weakness may also develop due to nerve compression.

Pathological fractures

When chondrosarcoma weakens the bone, it can cause the bone to break unexpectedly, even during normal daily activities. This condition is called a pathological fracture. Pathological fractures:

• Can sometimes be the first sign of the disease
• Result from the presence of a tumor that weakens the bone structure
• Can occur as a result of much less force or trauma than normal
• Require emergency medical intervention

In some cases, patients may suddenly experience a pathological fracture without any symptoms in their bones, and in this way they may receive a diagnosis of chondrosarcoma.

General symptoms: weight loss, fever, night sweats

In chondrosarcoma, in addition to local symptoms, general symptoms affecting the whole body may also be seen:

• Fatigue and loss of appetite
• Unintentional weight loss
• Unexplained fever and episodes of shivering
• Night sweats

However, such systemic symptoms are seen more rarely and usually appear in the advanced stages of the disease. Most patients with chondrosarcoma generally do not feel ill.

Symptoms may differ depending on where the tumor is located. For example, in chondrosarcomas located in the spine, neurological symptoms may occur as a result of the tumor pressing on a nerve root or the spinal cord. Similarly, in tumors located in the pelvic bone, hip pain and problems with bowel or bladder control may be seen.

Because the symptoms of chondrosarcoma can be confused with other diseases, it is important that you see a specialist physician if you have these complaints.

Types of Chondrosarcoma

Medical research has identified several types of chondrosarcoma with different characteristics. Each of these types has its own distinctive features, age groups in which it is commonly seen, and locations in the body. The four most common types of chondrosarcoma are described below.

Classic chondrosarcoma

Classic chondrosarcoma is the most common of all chondrosarcoma types and accounts for approximately 85% of cases. This type usually occurs in people over 50 years of age and is most often seen in the humerus (upper arm bone), femur (thigh bone) and rib bones.

Classic chondrosarcoma can be classified into different grades according to its histological features. This classification plays an important role in treatment planning. Classic chondrosarcoma is generally resistant to radiotherapy and chemotherapy, so the most effective treatment method is surgical intervention.

Dedifferentiated chondrosarcoma

Dedifferentiated chondrosarcoma accounts for approximately 10% of all chondrosarcomas and is a type that follows a more aggressive course than classic chondrosarcoma. In this type, a different type of cancer develops from within a more well-differentiated cartilage cancer.

This type is seen most often in people between 50 and 60 years of age, and its diagnosis is usually made in the femur (thigh bone) and pelvis (hip bone) region. Its symptoms include pain and swelling over the affected bone. Larger tumors can lead to bone fracture and inability to use the affected area.

Mesenchymal chondrosarcoma

Mesenchymal chondrosarcoma is a rare type that accounts for less than 2% of all chondrosarcoma types. Unlike classic chondrosarcoma, it is seen at younger ages (between 20 and 40 years) and usually occurs in the skull bones and the spine.

This type is characterized by the presence of primitive mesenchymal cells in areas showing high cell density, in addition to cartilage tissue. Because mesenchymal chondrosarcoma can be confused with Ewing sarcoma, making the correct diagnosis is important. In the treatment of this type, surgical excision may be applied together with chemotherapy and radiotherapy.

Clear cell chondrosarcoma

Clear cell chondrosarcoma is a type that accounts for less than approximately 2% of all chondrosarcomas and is seen mostly in people around 20 to 30 years of age. It takes its name from the clear appearance of the tumor cells under the microscope.

This type is characterized by slow growth and low metastatic potential. The most common sites are the epiphyseal regions of the long bones; the proximal femur (68%) and proximal humerus (23%) are especially frequently affected.

Although clear cell chondrosarcoma is known to be low grade, according to one study, 78.6% of patients were found to have an intermediate- or high-grade tumor. Its treatment is surgery with wide margins, and it is resistant to chemotherapy and radiotherapy.

In conclusion, recognizing the different types of chondrosarcoma is of great importance for accurate diagnosis and treatment planning. Each type has its own distinctive features and requires patient-specific approaches.

How Is Chondrosarcoma Diagnosed?

The diagnosis of chondrosarcoma is a comprehensive process carried out in multiple stages. To make an accurate diagnosis, the physician's examination, imaging methods and laboratory tests are evaluated together. Here are the stages of this process.

Physical examination and patient history

The diagnostic process begins first by listening to your complaints. Your doctor will:

• Assess the presence of pain, swelling or a mass
• Ask when the pain started and what makes the pain worse
• Find out whether there is a history of cancer in your family
• Obtain information about previous injuries and your general health

During the physical examination, the doctor checks the affected area for stiffness, swelling or limited movement. With manual examination (palpation), the structure of the mass and its relationship with the surrounding tissues are assessed.

X-ray, MRI and CT imaging

Imaging techniques play a very important role in the diagnosis of chondrosarcoma:

X-ray: This is the first imaging method used. It shows changes in the bone and determines the location and size of the tumor. On X-ray, chondrosarcomas are usually seen as calcifications with a "popcorn" appearance.

Magnetic Resonance Imaging (MRI): This is the technique that best shows the spread of the tumor within the bone marrow and soft tissue. MRI clearly demonstrates the borders and size of the tumor. On T2-weighted images, it appears as a lobulated, bright mass.

Computed Tomography (CT): By examining the bone structure in detail, it shows features such as bone destruction and calcification. It is also useful in assessing the presence of endosteal scalloping (hollows on the inner surface of the bone).

Types of biopsy and their importance

A biopsy is essential for a definitive diagnosis. With a biopsy, a sample is taken from the tumor tissue and examined pathologically. There are two types of biopsy methods:

• Needle biopsy: A tissue sample is taken with a thin needle under imaging guidance. It is less invasive and can be performed as an outpatient procedure.
• Open biopsy: A tissue sample is taken through a surgical procedure. It gives more reliable results, especially in cartilage tumors.

The planning and performance of the biopsy should be carried out by an orthopedic oncologist experienced in the treatment of chondrosarcoma. A biopsy that is not performed appropriately can cause the tumor to spread and reduce the patient's chance of recovery.

Advanced tests for staging

Once the diagnosis of chondrosarcoma is confirmed, advanced tests are performed to stage the disease:

• Lung CT: Because chondrosarcoma most often spreads to the lungs, a lung CT is performed to look for metastasis.
• Whole-body PET-CT or bone scintigraphy: These tests show whether the cancer has spread to other parts of the body.
• Bone scan: Used to identify metastatic foci in the bone.

Staging of chondrosarcoma is generally carried out using the Enneking classification or the American Joint Committee on Cancer (AJCC) classification. In this classification, the grade and size of the tumor, soft tissue extension and the presence of metastasis are evaluated.

The diagnosis and staging of chondrosarcoma should be evaluated by a multidisciplinary team that includes an orthopedic specialist, an oncologist, a radiologist and a pathologist.

How Is Chondrosarcoma Treated?

The fundamental approach in the treatment of chondrosarcoma is complete removal of the tumor. This treatment process should be planned and managed by a specialist orthopedic oncology team. The type, location and stage of the tumor and the patient's general health status play a decisive role in the treatment decision.

Surgical options for those undergoing chondrosarcoma surgery

Surgical treatment is the most effective method for chondrosarcoma, and the aim is complete removal of the tumor. The main goal of surgical intervention is to remove the tumor completely with safe surgical margins. In low-grade tumors, intralesional curettage and adjuvant therapies may be applied, whereas in high-grade tumors, wide en bloc resection is preferred.

The main surgical techniques used in patients with chondrosarcoma:

• Removal of the tumor with clear margins by wide resection
• Reconstruction with joint prostheses
• Bone graft applications
• Allograft-prosthesis combination

Limb-sparing surgery vs. amputation

First of all, limb-sparing surgery is the preferred method in all patients with chondrosarcoma. Thanks to advancing technology, many patients can be treated without the need for amputation.

However, in some cases amputation may be necessary. Amputation may be more appropriate especially when the tumor has spread over a very wide area and encases nerves and vessels, or in tumors that recur after incomplete surgery.

Use of radiotherapy and chemotherapy

Chondrosarcoma cells are generally resistant to chemotherapy and radiotherapy. These methods are not used routinely in the treatment of classic chondrosarcoma. However, in special situations they may be considered as supportive treatment:

• In tumors that cannot be completely removed surgically
• In the dedifferentiated and mesenchymal chondrosarcoma subtypes
• To reduce the risk of recurrence after inadequate resection
• For palliative purposes in metastatic disease

Rehabilitation process

Rehabilitation after treatment is very important for the patient to return to normal life. For those who have undergone chondrosarcoma surgery, the rehabilitation process includes the following stages:

• Early mobilization of the patient
• Maintaining and increasing range of motion in the affected joints
• Regaining muscle strength
• Training for return to activities of daily living

The rehabilitation process can last between 6 months and 1 year, depending on the extent of the surgery the patient has undergone and the areas affected. During this process, a team consisting of a physical medicine and rehabilitation specialist, a physiotherapist, an occupational therapist, a psychologist and other specialists takes part.

In summary, the treatment of chondrosarcoma requires a multidisciplinary approach and should be planned individually for the patient. Although surgical treatment is the main method, other treatment options should also be considered in special situations.

Chondrosarcoma in Summary

In conclusion, chondrosarcoma is a rare type of bone cancer that originates from cartilage cells. It generally occurs in people between 40 and 70 years of age and is more common in men. This disease can occur in bones in different parts of the body.

When you notice symptoms such as pain, swelling and stiffness, you should see a doctor without delay. Bone pain that increases especially at night and does not go away with rest can be an important sign.

For the diagnosis of the disease, a physician's examination and imaging methods such as X-ray, MRI and CT are used. A definitive diagnosis is made with a biopsy. At this stage, evaluation by a specialist team is very important.

The most effective method of treatment is surgery. Limb-sparing surgery is successfully performed today in many patients. The rehabilitation process after surgery is of great importance for recovery.

Undoubtedly, receiving a diagnosis of chondrosarcoma can be worrying. However, with advances in medicine, treatment options are steadily increasing. With the support of a specialist team and the right treatment approach, it is possible to maintain your quality of life.

Remember that early diagnosis is one of the most important factors in the success of treatment. For this reason, it is vital to go to a healthcare facility when you feel unusual pain or swelling in your bones.

Frequently Asked Questions

We have compiled the questions that patients most often wonder about chondrosarcoma. In this section, we will touch on some important points you need to know.

Grade 1 chondrosarcoma is a slow-growing, low-grade type of tumor. In this type, cellular changes are minimal and the risk of spreading to distant organs is quite low. However, the term "low grade" does not mean that the disease is insignificant. Over time, these tumors can grow, weaken the bone structure and cause loss of function. Surgical methods are generally preferred in treatment, and regular follow-up is of great importance.

In low-grade chondrosarcomas, the 5-year survival rate is approximately 83%. In high-grade types, this rate is around 53%. The prognosis varies depending on the grade and location of the tumor.

The following factors play a role in the development of chondrosarcoma:

• Advanced age (most patients are over 50)
• Sex (slightly more common in men)
• Benign bone tumors such as enchondroma and osteochondroma
• A genetic condition called multiple exostoses

The recovery process after surgery varies from person to person, but it generally lasts between 6 months and 1 year. Physical therapy and regular exercise can speed up the return to normal life. Every patient's recovery process is different, so it is important to follow your doctor's recommendations.

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