Chondrosarcoma- A Rare Cancer Not To Be Overlooked

4000 cases. That’s 4000 actual, living people screaming, kicking and fighting a rare kind of cancer. Well, that’s if you believe the Indian Cancer Society, anyway. You're at risk if you are an adult between the ages of 40-70.

Is it our lifestyle, you ask? That invites ailments like these, making them commonplace across our demographic. It all starts with a tumour on the impacted bone. Certain chondrosarcomas originate from precursor lesions, usually osteochondromas or enchondromas and are linked to

• Solitary osteochondromas and hereditary multiple exostoses

• Ollier disease

• Mafucci syndrome.

Chondrosarcoma is a rare type of cancer that has its origins in the bones but can occasionally happen in the soft tissue near bones. If you are unfortunate enough to be affected by this, you’ll notice it happens most often in the pelvis, shoulder and hip and rarely, only very seldom, in the spine's bones.

Usually, this isn’t too troublesome. Most chondrosarcoma grow slowly and might not cause many signs and symptoms initially, at least. Some rare varieties grow relatively fast and have a high risk of spreading to other areas of the body, which makes such cancers a chore to treat.

If you are afflicted with chondrosarcoma, surgery is the way to go. If that is something you don’t want, then radiation therapy and chemotherapy are your only options.

Chondrosarcomas develop in cartilage - which is the flexible connective tissue that safeguards your bones and joints. Healthcare providers might term this condition a sarcoma. Anywhere you have cartilage is fair game for this cancer, which usually forms in the

• Arms and shoulder blade

• Legs

• Pelvis

• Ribs

• Sternum (breastbone)

• They could also develop in benign bone tumours that turn cancerous.

There are several varieties of chondrosarcomas. The ones that develop in cartilage are named for the cells that comprise the tumours.

Conventional Chondrosarcoma

You’d see this most often, making up 85% of all chondrosarcomas. It usually impacts people in the 50-70 age band and develops in the femur, humerus (upper arm bone), and pelvis. Conventional chondrosarcomas don’t grow very fast and aren't more likely to spread (metastasize) than other types.

Dedifferentiated Chondrosarcoma

Dedifferentiated chondrosarcomas make up 10% of all chondrosarcomas. This variety usually comes up in adults aged 60 and older and grows quicker than most other chondrosarcomas. The swiftly-growing tumour usually develops in the humerus, femur or pelvic bones.

Dedifferentiated chondrosarcomas are named because some cells start out as typical chondrosarcomas, but some parts of the tumour morph into cells like those of a high-grade sarcoma. High-grade sarcomas are cancers that grow and spread super fast, including osteosarcoma, fibrosarcoma and undifferentiated pleomorphic sarcoma.

Clear cell chondrosarcoma

This variety accounts for 2% of all chondrosarcoma, usually impacting people aged 30 to 50. Don’t be surprised if it catches someone in their 20s as well. Clear-cell chondrosarcomas are usually found in close proximity to a joint in the arm or leg. They grow slowly and don’t really spread to other body parts.

Mesenchymal chondrosarcoma

This variety usually impacts adults aged 19 to 30. It usually develops in the spine, jaw, or ribs. Mesenchymal chondrosarcoma often grows fast, is more likely to spread than other chondrosarcomas, and is even more likely to recur post-treatment.

Extraskeletal myxoid chondrosarcoma is a type of mesenchymal chondrosarcoma. It forms in the soft tissue in the upper parts of the arms and legs but not in the bone or cartilage.

Usually, when it comes to chondrosarcoma, a doctor will enquire about your symptoms, including :-

• When you first saw them.

• Have they deteriorated

• How have they impacted your everyday life?

• A physical examination will be ordered, and they might ask you to carry out an imaging test or a biopsy.

Imaging tests

To diagnose chondrosarcomas, healthcare providers might ask you to do the following imaging tests :-

• XRay

• Computed tomography (CT) scan

• Magnetic resonance imaging (MRI) scan

• Positron emission tomography tomography (PET) scan

• Bone scan

• Biopsy

Your doctor might point you to an orthopaedist - a surgeon who specialises in conditions like bone cancer so that they can do a biopsy. The orthopaedist performs multiple biopsies to remove tissue from a suspicious lump so a pathologist can look at this tissue under a microscope. These varieties are:-

Fine-needle aspiration- The orthopedist will use a needle and a syringe to get the tissue sample.

Incisional biopsy - The specialist will cut into the tumour to glean a tissue sample.

Excisional biopsy - In this case, they remove the whole lump or section of tissue.

A pathologist will then examine this tissue sample for signs of cancerous cells. If they find them, they’ll pinpoint the tumour type and establish a tumour grade.

A tumour grade measures how fast a tumour has the potential to grow and spread. Chondrosarcoma grades range from one to three.

Grade I (Low Grade)

Such tumours, also known as atypical cartilaginous tumours (ACT), are characterised by mild cellular atypia, small chondrocytes and nuclei. They don’t grow too fast and are not likely to metastasise, either.

Grade II (Intermediate Grade)

Such tumours show increased cellularity and cellular/nuclear atypia compared to grade I. They are slightly more likely to spread.

Grade III (High Grade)

Such tumours are characterised by high cellularity, prominent nuclear atypia, and the presence of mitosis. They are most likely to metastasise.

When it comes to chondrosarcoma treatment, doctors usually suggest surgery to remove the cancer. Other treatments could be recommended, depending on the circumstances. Which options are the most suited for you depends on where the cancer is located, the rate of growth, whether it has grown to involve other structures, your holistic health, and your preferences.

Surgery

The main aim of surgery on chondrosarcoma is to remove the cancer and a margin of healthy tissue around it. The type of surgery you will undergo will depend on the location of the chondrosarcoma.

Options might include -

Scraping the cancer from the very bone. Small and slow-growing chondrosarcomas in the arms and legs are occasionally treated with a procedure to scrape the cancer cells from the very bone.

The surgeon might apply cold gas or a chemical to exterminate any remaining cancer cells. If necessary, the bone can be repaired with a bone graft or bone cement.

Cutting away the cancer and some healthy tissue around it

Most chondrosarcomas require a procedure to cut away some more of the bone in order to get rid of all the cancer. For chondrosarcoma in an arm or leg, amputation might be necessary.

Surgeons do everything possible to prevent the same, however. For instance, the surgeon might remove the affected bone and replace it or reconstruct it (limb salvage surgery).

Radiation Therapy

In radiation therapy, high-powered energy beams from sources like X-rays and protons kill cancer cells. During radiation therapy, you lie supine on a table while a machine moves around you, directing radiation to different precise points on the body.

Radiation is recommended for chondrosarcomas located in places surgery can’t reach or if the cancer can’t be removed completely during surgery. Radiation might also be used to curtail cancer that spreads to other body parts.

Chemotherapy

Chemotherapy depends on drugs to kill cancer cells. It is not often used for chondrosarcoma because this type of cancer usually doesn’t respond to chemotherapy. However, some fast-growing varieties of chondrosarcoma might respond to this treatment.

Chondrosarcoma cannot be prevented because researchers are unsure how it happens. However, research suggests that people with certain inherited disorders have a heightened risk of developing it. Speak to a doctor about your family medical history so they can gauge your risk and give you the best suggestions for monitoring your health.

What Are the Options for a Cure?

In certain cases, surgery to remove small, slow-growing conventional chondrosarcoma tumours might help cure the condition. Depending on the variety of chondrosarcoma, adding chemotherapy might also help.

Living With Chondrosarcoma

Living with chondrosarcoma usually involves recovering from treatment. Chondrosarcoma is generally treated with surgery that could include removing a limb or section of a limb to remove the cancer. Once you recover from surgery, you’ll need to commence rehabilitation to help you adjust to using a prosthetic limb.

You might need some assistance adjusting to the other changes, like how you go about your daily life or changes in your appearance. Your doctor will give you suggestions for physical, occupational and mental health therapies that could help you deal with these challenges.

Being diagnosed with a rare bone cancer like chondrosarcoma could be worrisome; however, don’t worry because this is also the first stop of what could be a really long road to recovery.

Often, that road begins with surgery to remove the cancer and rehabilitation to recover from surgery and other treatments. While this cancer is treatable, it is a life-changing experience.

You might need assistance to cope with the physical and emotional challenges. Speak to your doctor about the issues you’re facing. They will know what you’re going through. As you start on your way to recovery, they’ll be with you every step of the way.

FAQs

How does chondrosarcoma happen?

Researchers are none the wiser about how chondrosarcoma happens. Genes could be anybody’s guess. Well, it doesn’t matter how it happens. What happens is whether you are on the road to recovery.

What is the best course of treatment for chondrosarcoma?

Surgery is the best way forward if you have chondrosarcoma. The surgeon will remove the cancer from the body as much as possible.

What are the grades of chondrosarcoma?

Chondrosarcoma can be differentiated into Grade 1 (Low Grade), Grade II (Intermediate Grade) and Grade III (High Grade)

What are the symptoms of chondrosarcoma?

Common symptoms of chondrosarcoma include:

• Pain in the affected bone or joint may worsen over time.

• Swelling or a lump in the affected area.

• Limited mobility or difficulty moving the affected limb.

• In some cases, a fracture in the bone is due to the weakened structure caused by the tumour.

How is chondrosarcoma diagnosed?

Diagnosis usually involves:

• Physical Examination: To check for lumps or tenderness in the bones.

• Imaging Tests: X-rays, CT scans, and MRIs to locate and assess the tumour.

• Biopsy: A sample of the tumour is taken and examined under a microscope to confirm if it's cancerous.