This information has been written for patients, their families and friends and the general public to help them understand more about a form of primary bone cancer known as chondrosarcoma. This section will detail what chondrosarcoma is and how chondrosarcoma is diagnosed and treated.
For a downloadable source of this information, please view our 'Downloadable Information Materials' page to view all of our fact sheets.
Sarcomas are cancers that start in connective tissue, which are the body parts that have a supporting role in the body. The bones, cartilage, muscle and blood are all types of connective tissue.
Chondrosarcoma is a rare cancer that most often forms in the bone, but can also very rarely appear in the soft tissue.
Chondrosarcoma is the most common primary bone cancer in adulthood, and the second most common primary bone cancer overall. It makes up around 25% of all malignant bone cancer cases. The tumour is made of cells that produce cartilage.
Chondrosarcoma can develop in any part of the body but the most common sites are the: pelvis, rib cage, arms (upper arm or humerus), shoulder blades and legs (proximal femur in the thigh and the tibia in the shin). Chondrosarcomas can also be found in the spine or skull but this is extremely rare.
Also extremely rare is a type of chondrosarcoma called 'extraskeletal chondrosarcoma', which does not form in bone. Instead, it forms in the soft tissues of the upper part of the arms and legs. Chondrosarcomas that originate in internal organs have also been seen.
The majority of chondrosarcomas are slow growing and do not spread, but occasionally the cancer cells can spread (metastasise) away from the bone in which they start. The likelihood that chondrosarcoma will spread depends on the type of chondrosarcoma and the grade. If chondrosarcoma does spread from its site of origin, it usually spreads to the lungs.
Chondrosarcoma can be described using different classifications. This helps doctors decide how best to treat the tumour.
Tumours are classed as primary or secondary.
- Primary chondrosarcomas start from cells in a healthy bone.
- Secondary chondrosarcomas arise from a benign tumour that is already present in the bone.
These benign tumours include enchondroma and osteochondroma.
The location of the tumour in the bone. Chondrosarcomas can be either:
- Central, meaning inside the bone cavity.
- Surface, meaning on the outer surface of the bone and sometimes described as periosteal.
Peripheral chondrosarcomas are surface chondrosarcomas that are secondary to osteochondroma.
When the tumour is examined under a microscope, differences can be seen between some tumour types. This classification is called the 'histological variant'.
Chondrosarcoma should not be confused with 'chondroblastic osteosarcoma'. This is a bone-forming tumour, but the tumour cells also form cartilage. The treatment for chondroblastic osteosarcoma is the same as for osteosarcoma, not chondrosarcoma.
There are four types of primary chondrosarcoma. They range from slow-growing non-metastasising lesions, to very aggressive metastasising tumours.
Most chondrosarcomas (around 75% of cases) are called 'conventional chondrosarcomas'.
Conventional chondrosarcomas are further classed based on their appearance under a microscope. Pathologists examine the cells in a biopsy of the tumour and examine the size of a structure within the cells called the nucleus. The pathologist assigns the grade of the tumour to say how different the cells look from healthy cells
- Low grade tumours (grade 1)
Most cells look like normal cartilage, some cells may have two nuclei.
- Intermediate grade tumours (grade 2)
Cell nuclei are misshapen, fragmented, or bigger than in low grade tumours. Intermediate tumours are treated in the same way as high grade tumours.
- High grade tumours (grade 3)
Cells of the same type have different shapes, lots of cells are in the process of division (mitosis), giant cells and many dead cells (necrosis) can be seen.
Grade 1 tumours are less aggressive than grades 2 and 3 tumours, meaning that grade 1 tumours are easier to treat. Most patients have low or intermediate grade conventional chondrosarcomas although rarely, conventional chondrosarcomas can be high grade.
Conventional chondrosarcomas can affect any bone. Most conventional chondrosarcomas arise from inside the bone; however, around 15% arise from cells on the surface of the bone. Conventional chondrosarcomas are equally likely to appear in the central part of skeleton as in the bones of the limbs. The thigh bone (proximal femur) is the most frequently affected followed by upper arm bone, distal femur, and ribs.
- Around 10% of chondrosarcomas are dedifferentiated chondrosarcoma.
- They are high grade and very aggressive.
- The most common sites affected are the thigh (femur) and pelvis.
- This is a very agressive form of chondrosarcoma. It is extremely rare and accounts for less than 2% of chondrosarcomas.
- It can occur anywhere in the body - either in the bones or in soft (non-bony) tissues.
If the tumour first appears in non-bony tissue it is described as 'extra-skeletal', which means 'outside the bones'.
- Around 70% of mesenchymal chondrosarcomas originate in bone, while 30% are extra-skeletal.
- Cells of the tumour may look like cells of Ewing's sarcoma, a different bone tumour.
Clear Cell Chondrosarcoma
- These low grade tumours are extremely rare, accounting for less than 2% of chondrosarcomas.
- This type of chondrosarcoma tends to occur in the long bones of the arms and legs.
- The inside of the cells (cytoplasm) appears clear/ empty under the microscope.
The type of treatment a patient receives will depend on which type of chondrosarcoma is diagnosed.
The main treatment for chondrosarcoma is surgery to remove the tumour. Radiotherapy is sometimes used, and much more rarely chemotherapy might be used.
Chondrosarcoma is the most common form of primary bone cancer affecting all age groups.
Chondrosarcoma can affect people of all ages but is mostly found in adults between the ages of 30 and 60 years. 80% of cases are in people aged 40 years or older.
In the UK there are around 190 new cases of chondrosarcoma diagnosed each year. In The Republic of Ireland there are around 12 new cases of chondrosarcoma diagnosed each year.
The incidence rate in England is around 2.9 people affected per 1 million people of the population.
The different types of chondrosarcoma affected different individuals:
- Is mostly found in adults over the age of 50 years.
- Affects males slightly more than females.
- Accounts for 75% of all chondrosarcoma cases.
- The average age at presentation is between 50 and 60 years.
- Males and females appear to be affected equally.
- This can occur at any age, but it has peak incidences in teens and young adults.
- Males and females appear to be affected equally.
Clear cell chondrosarcoma
- This can occur at any age, but peak incidence is in 30s and 40s.
- Males are more commonly affected than females (~ 2.5: 1).
Presentation: doctors talk about 'presentation' when they mean 'symptoms' and 'clinical signs'.
Symptoms: these are the feelings and signs that a patient or a parent/carer sees or feels, which signal that the patient is ill.
Clinical signs: these are what the doctors may see during a physical examination, giving doctors clues about what is wrong with the patient.
The most commonly-reported symptoms of chondrosarcoma are:
- Localised pain. This can be dull in nature, occurring when a patient is at rest, and may become worse at night. Pain may also become progressively worse.
- Local swelling.
- Walking with a limp or having restricted movement of a joint (if near the affected bone).
These symptoms can be present alone or in combination. These symptoms are often of long duration, possibly several months or years. The average duration of symptoms before diagnosis is thought to be between 1 and 2 years.
20-30% of chondrosarcomas are painless and may only be found when a patient suffers with a fractured bone caused by a mild injury, such as from a minor fall or accident. The fracture can happen where the bone has been weakened by the tumour. This is known as a pathological fracture.
Around 50% of patients with spinal chondrosarcoma will experience neurological symptoms, which result from the tumour pressing on the nerves in the spine. This can include pain, numbness or tingling, muscle spasms or even muscle weakness.
The symptoms of chondrosarcoma are all common to other conditions, which makes it hard for doctors to reach the correct diagnosis.
The most common clinical signs are:
- A mass that can be felt (palpable) when undergoing physical examination.
- Broken bone (fracture).
There has been a lot of research into possible causes of chondrosarcoma but the underlying cause remains unknown in most cases.
Chondrosarcoma is not contagious. It cannot be passed on to another person by exposure to a chondrosarcoma patient.
- It is known that cells contain important information in their chromosomes called genes, which help them to divide and grow normally. Damage to these genes in one single cell can cause the cell to behave differently and grow abnormally, which can then lead to development of cancer.In chondrosarcoma, there is damage to the cell's genetic information such as abnormalities of genes (mutations) called tumour suppressor genes and oncogenes as well as genes which control the copying of the cell's DNA. We do not know why the damage occurs in most cases.In some young patients we know that chemotherapy or radiotherapy for another tumour can damage the DNA enough to cause chondrosarcoma
- Some recent research has identified a pair of genes that are frequently damaged in chondrosarcoma tumours. Researchers looked in cells from many different chondrosarcoma tumours and found that two genes, called IDH1 and IDH2, were damaged (mutated) in many tumours. These genes are also found to be damaged in some other cancer types. We don't know why these genes get damaged but we do know that the damage is not inherited.
- A small proportion of patients are known to have inherited damaged genes from their parents. This is known as a genetic predisposition, which means they have an increased risk of developing different types of cancer, including chondrosarcoma.
Doctors and scientists all over the world are involved in research to try to understand the difference between normal bone cells and chondrosarcoma cells. This may enable them to find treatments that can target the abnormal chondrosarcoma cells rather than normal cells in the body.
The risk factors for chondrosarcoma
Although doctors do not yet fully understand what causes chondrosarcoma, there are several factors that put people at a higher risk. A risk factor is something that can increase the chance of getting an illness.
General risk factors
Males have a slightly higher risk compared to females.
Most chondrosarcomas develop in people over the age of 40.
- Underlying bone diseases
Bones which already have something wrong with them (underlying bone abnormalities) are at a higher risk of gettingn chondrosarcoma. These diseases are very rare:
- Paget's Disease is a disease that makes bones painful and encourages bone cells to divide more rapidly.
- Ollier's Disease is a condition in which benign tumours in the bones cause the affected bones to swell. The condition usually presents before the age of 10. Patients with Ollier's Disease have around a 20-25% chance of developing chondrosarcoma. Many Ollier's Disease patients have mutations in IDH1 or IDH2 genes.
- Maffucci Syndrome is a rare disorder that gives people with this condition a 20-25% risk of developing chondrosarcoma. Many Maffucci Syndrome patients have mutations in IDH1 or IDH2 genes.
Inherited risk factors
These are risk factors that people inherit from their parents and have from birth (hereditary). They can be passed on from one or both parents. The risk is passed to the child through the parent's genes. So, just as children inherit features such as hair or eye colour from their parents, a very small number can inherit a risk that will increase their chance of getting chondrosarcoma.
Certain hereditary conditions may make people more susceptible to chondrosarcomas:
- Multiple hereditary exostoses (hereditary skeletal disorder)
Patients with this condition have a 0.6% to 2.8% risk of developing chondrosarcoma.
- Wilm's tumour (kidney tumour)
In extremely rare cases, chondrosarcomas have been seen as secondary tumours from a primary Wilm's tumour.
Environmental risk factors
- Treatment by radiation or chemotherapy for a pre-existing condition
People who have received radiotherapy for cancer before have a slightly higher chance of developing chondrosarcoma.
Going to the doctor
People report a variety of experiences when they seek medical advice about their symptoms. Most people with worrying symptoms go to their General Practitioner (GP). Some people are referred quickly for further tests or a second opinion, but often patients have to return to their GP at least three or four times
Primary Bone Cancers are very rare and many GPs will never come across a case in their whole career. The symptoms of chondrosarcoma are common to other disorders and can be initially diagnosed as other less serious conditions at the first visit.
The Bone Cancer Research Trust is trying to find ways to make the time between the start of symptoms and getting the diagnosis much shorter. This is not a simple task and currently there is only a small amount of research on the subject. However, we are participating in a national project to look at ways of reducing the length of time it takes to reach a diagnosis.
Some patients go to their local hospital emergency department (A&E) or other health care centres. There is no scientific evidence available to examine whether patients presenting to their A&E are diagnosed any faster than patients who go to their GP.
For GPs in England and Wales there are NICE (National Institute of Clinical Excellence) guidelines to help doctors in diagnosing primary bone cancers, including chondrosarcoma:
If a GP suspects a primary bone cancer, they will normally ask for an x-ray of the bone or refer the patient for a specialist opinion. This is normally done at a local hospital or clinic. They may also ask for some blood tests to look at the patient's general health.
If the x-ray shows that that the patient may have a primary bone cancer, then more tests are necessary. Some of these may also be done in a local hospital but patients should be referred to a Bone Cancer Centre early on during his process for the completion of these tests.
Going to a Bone Cancer Centre for more tests
Once an abnormality is found in a bone that suggests the possibility of cancer, the patient will be referred to a Bone Cancer Centre.
Bone Cancer Centres are specialist hospital centres. They have a group of healthcare specialists who are experts in the diagnosis and treatment of bone cancer.
In England, there are currently five Bone Cancer Centres, which specialise in the diagnosis and treatment of primary bone cancers. These centres are at Birmingham, Newcastle, Oswestry, Oxford and Stanmore.
In the Republic of Ireland, there are no specific Bone Cancer Centres. Patients are initially seen in their local hospital and subsequently referred to specialist hospitals in Dublin or Cork for further tests and, if necessary, for treatment.
Patients in Wales usually travel to Oswestry or Birmingham for these specialist tests.
In Scotland there are five sarcoma centres and so patients travel to one of these centres for diagnosis if primary bone cancer is suspected. These centres are in Edinburgh, Glasgow, Inverness, Aberdeen and Dundee.
In Northern Ireland, patients are usually seen in Belfast.
For a full list of locations patients may be referred to in order to confirm a primary bone cancer diagnosis please click here
Specialists in many different areas of medicine at hospitals in Ireland and at Bone Cancer Centres and the Regional Cancer Centres in the UK work together as a 'Multidisciplinary Team' (MDT). The members of the Multidisciplinary Team work together to diagnose the patient's condition.
The MDT who will look after the patient during investigation (tests) and diagnosis will include:
- Specialist bone sarcoma surgeons.
- Specialist sarcoma oncologists (oncologists are doctors that look after people with cancer).
- Specialist sarcoma pathologist (pathologists are doctors that use laboratory techniques to diagnose disease).
- Radiologists (doctors that diagnose disease and conditions from looking at x-rays, or scans).
- Cancer Nurse Specialists (specially trained nurses).
What tests are done?
When a person is referred to a Bone Cancer Centre, further tests will be done to find out more and to confirm whether the patient has bone cancer, and if so what kind of tumour it is.
Patients are likely to have several different tests, including:
- X-rays are taken of the bone, including the joints above and below, and the radiographs (x-ray pictures) are studied. These x-rays may show swelling around the bone or areas of abnormal bone growth.
A chest x-ray is sometimes taken to show whether the cancer has spread to the lungs.
- Blood tests involve taking a small sample of blood from a vein using a needle. The sample of blood is tested in laboratories to check a patient's general health and for levels of certain substances or chemicals in the blood. These include:
- Blood chemistry (Urea and Electrolytes) is checked to examine the levels of normal salts and urea and creatinine, which are waste products. This test can give clues to how well the kidneys are working
- Full blood count (FBC) counts the numbers of different types of blood cells in the patient's blood at that time. These include:
Red blood cells - which carry oxygen in the blood.
White blood cells - which are essential to the immune system (and totals of each type).
Platelets - which are essential to the making blood clots and scabs.
Levels of haemoglobin - which is found in red blood cells.
- Liver function tests (LFTs) to see how the liver is working.
- Erythrocyte Sedimentation Rate (ESR) is a test to look for signs of inflammation.
- C-Reactive Protein (CRP) levels are tested as CRP levels increase in inflammation.
- Alkaline phosphatase (ALP) levels are measured in patients with suspected osteosarcoma.
MRI stands for 'magnetic resonance imaging'. This type of scan is similar to a CT scan but magnetism and radio waves are used instead of x-rays to build up a very detailed 3-dimensional image.
An MRI scan of the entire bone is used to gain more information about the tumour in the bone.
The scanner is doughnut shaped; there is a short tunnel, which a motorised bed moves through during the scan.
The MRI scanner can be quite noisy - some machines have a CD player so that patients can listen to their choice of music during the scan.
Sometimes an injection of a special dye, known as a contrast agent may be needed. This makes certain tissues show up more clearly and with greater detail on the scan. The results of the scan will be examined by a radiologist and a report will be produced.
CT stands for 'computerised tomography'. They may also be called CAT scans, which stands for 'computerised axial tomography'.
The scanner takes x-rays from many different angles and a computer builds up a 3-dimensional picture of the body in great detail. The pictures show cross-sections of the inside of the body.
CT scanning of the lungs shows up any secondary tumours where the cancer may have spread (metastases). It is used if the MRI scan results have not been able to confirm the diagnosis of osteosarcoma. CT scans are also used to help doctors understand more about the exact location and size of the bone tumour.
A CT scanner looks like a large doughnut with a bed for the patient to lie on. The bed moves slowly through the hole of the doughnut while the machine takes the pictures.
Before the scan, patients may be given a contrast medium - usually injected into a vein. This helps to improve the image of some particular tissues, and it can also help the radiologist tell the difference between blood vessels and other structures.
The results of the scan will be examined by a radiologist and a report will be produced.
PET stands for 'positron emission tomography.' Not all hospitals have PET scanners but they are increasingly being used in the diagnosis of cancer.
PET scans can examine the whole body, rather than a specific area. They can also detect how well treatments are working.
Before the scan, a small injection of radioactive glucose (a radiotracer) called fluorine18 will be given. Glucose is the fuel that cells use for energy. Cells that are very active such as cancer cells will take up more of this radioactive glucose than less active cells.
The tracer will take around an hour to spread around the body. During the scan, which can last about an hour, the patient lies on a bed and the scanner passes over them. The scanner detects where the radiation is concentrated and produces images.
When the body breaks down the radioactive glucose, particles called 'positrons' are released or emitted, the PET scanner detects the energy from the positrons and shows up as a 3-D image on a computer screen. Areas of high positron concentrations show up as a different colour and brightness on the image compared to areas of low positron concentration.
The results of the scan will be examined by a radiologist.
Bone scans are used to look for abnormalities in bones. Some patients may have bone scans if chondrosarcoma is suspected, but newer technologies such as PET scans are taking over.
- A tiny amount of radioactive substance (radionuclide) is injected into the patient's blood, which is then taken up by the bones fairly quickly (~2-4 hours).
- During the scan the radioactivity is detected by a specialised camera called a gamma camera. The radioactivity will collect more at areas of high activity (breakdown and repair) in the bone. These areas can be caused by a tumour.
- The areas of high activity picked up by the gamma camera are known as 'hot spots.'
- The scans are usually carried out in hospital nuclear medicine departments.
- Patients will need to drink lots of fluids before the scan to help the radioactive substance travel to the bones quickly. The scanner looks a bit like a CT scanner (doughnut shape). Patients lie on the bed, which travels through the doughnut.
- The results of the scan will be examined by a radiologist who will write the results in a report, which may take a few days to produce. Following the scan, the radionuclide will be passed completely from the body in the urine within 24 hours.
A bone biopsy is a specialised procedure that should only be performed by a specialist in orthopaedic surgery or sarcoma radiology. These biopsies should only be performed at a Bone Cancer Centre.
- A biopsy involves taking a small sample of a lump or tumour. A pathologist (a doctor who uses laboratory techniques to diagnose disease) looks at this sample under a microscope. This enables them to work out what type of cells the tumour is made up of and whether or not it is cancerous. This is known as the 'histology'.
- The biopsy sample can be taken using a specialised syringe-type needle, which is called 'needle biopsy', or during surgery, which is called 'surgical biopsy'. In both cases, a small amount of the lump is taken. Sometimes a scan (ultrasound or CT) is performed at the same time to make sure that the biopsy is taken from the right place.
- The biopsy is studied by pathologists. This takes time and so the results of a biopsy can take a week or more.
The biopsy enables doctors to diagnose chondrosarcoma. This is important because other conditions (including non-cancerous conditions) can look like chondrosarcoma on x-rays and scans. This is known as a differential diagnosis.
What other diagnoses might be made if it's not chondrosarcoma?
After doing some or all of these tests the medical team will be able to make a diagnosis.
If the tests show that the patient does not have chondrosarcoma then there are a number of other conditions that it might be:
- Enchondroma; a benign cartilage tumour.
- Fibrous dysplasia; a bone disease, in which normal bone is replaced by weak bone-like tissue.
- Osteomyelitis; infection of the bone.
What if the tests confirm that it is chondrosarcoma?
At this point, most patients will be referred to their Regional Cancer Centre where the medical team will design the best treatment plan for the patient and treatment will start.
The hospital should be a specialist Bone Cancer Centre or a Children's and Young People's Cancer Centre if the patient is a young adult or a child.
How is a treatment plan decided?
Once an abnormality is found in a bone that suggests the possibility of cancer, the medical team will carry out tests in order to answer two essential questions:
- What kind of cancer is this?
- Where in the body is the cancer?
The type of treatment and how likely it is that treatment is successful depends on the answers to these two questions.
What kind of cancer is this?
X-rays and scans may show that the patient is very likely to have chondrosarcoma, but doctors cannot be fully certain without taking a small piece of the suspected tumour, called a biopsy. The biopsy goes to a special laboratory so that it can be examined under a microscope. By looking at the cells in the biopsy sample under the microscope, an expert pathologist can make the diagnosis of chondrosarcoma.
Pathologists are doctors who use laboratory techniques to diagnose disease. The pathologist can also find out the 'subtype' and 'grade' of the cancer. The 'grade' describes how different the cells of the tumour look when compared to normal cells and helps predict how quickly the cancer may grow or spread to other parts of the body.
Where in the body is the cancer?
X-rays and scans help the doctors to see the size and exact place in the body of the tumour and to look for evidence if the cancer has spread to any other parts of the body. This is known as 'staging' of the cancer.
In a small number of patients with chondrosarcoma, the scans may show that the cancer has spread, usually to the lungs. Cancer that has spread away from the primary site is called 'secondary cancer' and doctors called these secondary tumours 'metastases' (meh-TAS-tuh-sees). In most chondrosarcoma patients, there is no sign that the cancer has spread by the time they begin treatment.
Who is involved in deciding the treatment for chondrosarcoma?
Specialists in many different areas of medicine at hospitals in Ireland and at Bone Cancer Centres and the Regional Cancer Centres in the UK work together as a 'Multidisciplinary Team' (MDT).
The Multidisciplinary Team will work out the details of the treatment needed. The MDT consists of:
- Specialist bone sarcoma surgeons
- Specialist sarcoma oncologists (oncologists are doctors that look after people with cancer)
- Specialist cancer nurses
- Specialist sarcoma pathologist (pathologists are doctors that use laboratory techniques to diagnose disease)
- Radiologists (doctors that diagnose disease and conditions from looking at x-rays, or scans)
- Physiotherapists and occupational therapists will help with rehabilitation (rehab) after surgery
- Social workers and psychologists will help with patients' emotional, social and educational needs
When patients arrive at their Regional Cancer Centre, more tests will be carried out to show how well organs such as the kidneys, heart, liver and ears are working. Tests may include blood tests, echocardiograms (heart) and an audiogram (hearing test). The measurements from these tests show doctors if these organs are working normally. This gives the doctors a 'baseline' to compare how well these organs are working as treatment goes on, in cases where patients are given chemotherapy.
Treatment for chondrosarcoma
The main and still the most successful treatment option for chondrosarcoma is surgery to remove the tumour (surgical resection).
For higher-grade tumours, such as dedifferentiated or mesenchymal chondrosarcomas, which are at higher risk of recurrence and spread (metastasis); adjuvant therapies such as radiotherapy, chemotherapy or proton therapy may be used.
The aim of surgery is to remove every last cell of the primary tumour. This prevents the tumour from growing back later, which is called a local recurrence, or spreading to another part of the body, which is called metastasis. The surgeons aim to remove the entire tumour safely and at the same time try to keep the body working as normally as possible.
Surgery for low grade chondrosarcoma
Low grade chondrosarcomas can be treated by different surgical techniques: either a wide resection (which involves cutting out the tumour plus the tissue directly next to the tumour) or a procedure called curettage (which involves scraping the tumour cells out of the bone) followed by adjuvant treatment such as cryosurgery (which means using liquid nitrogen to freeze out any remaining cancer cells), or high speed burring of the cavity.
Reconstruction of the bone defect depends on which bone is involved and the preference of the surgical team, but may involve the use of:
- Allograft bone - using donated bone to repair the patient's bone after surgery.
- Autologous bone - this is when bone is taken from another part of the patient's body to replace the bone that has been removed during surgery.
Surgery for high grade chondrosarcoma
The type of surgical procedure will depend on the size and location of the tumour. The aim of the surgery, where possible, is to remove the entire tumour along with a wide margin of the surrounding healthy tissue (wide resection). For some tumours it may be possible to remove the tumour without having to reconstruct the bone and losing too much function.
For important bones such as the femur or pelvis, the surgery may include complex site-appropriate reconstruction of the bone using:
- Endoprosthetics - metal implant in the bone or a false joint.
- Allograft bone - using donated bone to repair the patient's bone after surgery.
- Autologous bone - this is when bone is taken from another part of the patient's body to replace the bone that has been removed during surgery.
Removing tumours can be very complicated, and requires very careful individual planning for each patient.
If the tumour is in the spine or base of the skull, it is much more difficult to remove without damaging the spine or brain. In these cases radiotherapy may be used where it is not possible to remove the whole tumour surgically.
If there is evidence that the tumour has spread to other parts of the body then an oncologist and surgeon may want to think about the possibility of removing the secondary cancers by surgery.
Radiotherapy is not very effective as a treatment in the majority of patients with chondrosarcoma.
However, there are times when radiotherapy is used:
- When chondrosarcomas are difficult to remove surgically
- As a treatment for mesenchymal or dedifferentiated chondrosarcoma
- To decrease pain and discomfort when a patient's cancer is advanced and unable to be cured. This is known as 'palliative radiotherapy'.
Chemotherapy is not usually used as a treatment for chondrosarcoma, as the tumours are resistant to the chemotherapy drugs, meaning that chemotherapy is not effective.
However, chemotherapy is sometimes used for patients with mesenchymal or dedifferentiated chondrosarcoma, as these tumour sub-types seem to respond better than conventional chondrosarcomas. The chemotherapy drugs that can be used in this treatment include doxorubicin and cisplatin.
Although these drugs are sometimes used, it is not yet clear whether these treatments have an effect on outcome. This is largely because the very small numbers of these forms of chondrosarcoma make it difficult to study.
The doctor (oncologist) is the best person to talk to about treatment choices. The doctors will also tell patients what to expect from the treatment. Treatment of cancer involves patients and their doctors working together to find a care or treatment plan that fits the patient's needs.
Proton therapy is a new kind of radiotherapy that allows a greater dose of radiation to be delivered safely without affecting surrounding tissues and so this may provide an effective and safe treatment.
In addition, better focusing of the radiation used in radiotherapy (spot scanning) allows the use of more intense radiation without increasing the toxicity to the healthy issues in the body. This has recently been tested in patients with chondrosarcoma at the base of the skull.
Two proton therapy centres are being built in the UK, one in London and one in Manchester. These facilities will be open in 2018. Until then, patients in the UK are able to travel to Switzerland or the USA for proton therapy, if the MDT recommends this type of treatment. Government funding is available to pay for patients and carers to travel abroad for this treatment.
Where will treatment take place?
Treatment for chondrosarcoma can take place at different hospitals around the UK and Ireland. For patients whose nearest specialist hospital is too far away, a 'shared care' arrangement with a closer hospital might be set up.
This means that the specialist hospital recommends a treatment plan, which is used to treat the patient at a hospital closer to home.
England and Wales
Diagnosis and surgery should take place in one of the five Bone Cancer Centres (see the map below):
- North of England Bone and Soft Tissue Tumour Service (Newcastle upon Tyne Hospitals NHS Foundation Trust)
- Nuffield Orthopaedic Centre NHS Trust (Oxford)
- Royal National Orthopaedic Hospital (Stanmore, Middlesex)
- The Robert Jones and Agnes Hunt Orthopaedic and District Hospital NHS Trust (Oswestry)
- The Royal Orthopaedic Hospital, Bristol Road South (Northfield, Birmingham)
Patients are treated at one of the five Sarcoma Centres that are part of the Scottish Sarcoma Network. These hospitals are in Aberdeen, Dundee, Edinburgh, Glasgow, and Inverness. Patients visit one of these five Sarcoma Centres for chemotherapy or radiotherapy treatment. For surgery, primary bone cancer patients are seen in Glasgow, Edinburgh or Aberdeen.
Republic of Ireland
Most patients aged under 16 receiving chemotherapy for osteosarcoma are treated at Our Lady's Hospital, Crumlin, Dublin.
Patients aged 15-19 are treated at Mater Misercordiae Hospital, Our Lady's Hospital, Crumlin and Waterford Regional Hospital.
Patients aged over 20 are treated at Mater Misercordiae Hospital, Our Lady's Hospital Crumlin, Sligo General Hospital, Cork University Hospital, Waterford Regional Hospital, St Vincent's Hospital and Mercy Hospital.
For surgery, most patients in the Republic of Ireland (all ages) go to St. Marys Orthopaedic, Cappagh. For radiotherapy most patients attend St Luke's and St Anne's Hospital, Dublin. However, some patients may also attend other hospitals in Dublin and Cork.
Red stars: Specialist Children's Cancer and Leukaemia Centre
Blue stars: Bone Cancer Centre
Green stars: Children and Young People's Integrated Cancer Service
Purple stars: Teenage Cancer Trust Unit
Yellow stars: Scottish Sarcoma Network Hospital
Please see our full list of centres providing treatment for primary bone cancer here.
Research into the biology of chondrosarcoma can reveal targets for novel treatments
Research is needed to find better treatments for chondrosarcoma because chemotherapy and radiotherapy are not normally effective on these tumours. Also we don't know what makes chondrosarcoma recur (come back again) in some patients, or metastasise (spread to other organs).
If scientists and doctors can better understand why chondrosarcoma is resistant to chemotherapy and radiotherapy, their research may reveal new targets for treatment or adjuvant treatments to surgery.
One area of research that is currently underway is a series of investigations into a gene network called the "hedgehog pathway" (named after Sonic the Hedgehog, a computer game character). This network of genes is part of the machinery that works inside cells to control cell division. Researchers have found that chondrosarcoma cells in the laboratory stop growing when the hedgehog pathway is switched off. A phase 2 clinical trial of a new drug that targets the hedgehog pathway in chondrosarcoma has recently been carried out in the USA and if the outcomes from this trial are positive then wider trials may follow.
Scientists do have some understanding why chondrosarcoma is resistant to chemotherapy and radiotherapy.
Chemotherapy and radiotherapy target rapidly dividing cells. The majority of chondrosarcomas are low grade (grade 1) and are therefore relatively slow-growing; this makes them resistant to chemotherapy and radiotherapy.
In addition, some chondrosarcomas express a gene called 'P Glycoprotein,' which is normally found in intestinal tissues, and in the brain. P Glycoprotein exists in healthy tissue to pump poisons out of cells. If P glycoprotein gets switched on in cancer cells it pumps chemotherapy drugs out of the tumour, rendering the drugs ineffective.
P glycoprotein is effective against many different drugs and so is a big barrier to cancer treatment.
Chondrosarcomas also tend to have a very poor blood supply meaning that it is difficult for drugs to reach the tumour cells.
Radiotherapy resistance (radioresistance) in chondrosarcoma is caused by the loss of certain genes that are the targets of radiation.
In normal cells, radiation damage to a cell causes very reactive molecules called 'reactive oxygen species' or ROS to be produced. These ROS can damage or break the cell's DNA, which damages the cell's genes. DNA damage in the cell results in the activation of 'tumour suppressors,' which are proteins that either repair the damage or start cell suicide (apoptosis) to prevent the genetic damage being passed on when the cell divides. If any of the functions that are needed to recognise the damage and act on it are missing in the cancer cell then they will be radioresistant (resistant to radiotherapy).
Loss of particular genes known as tumour suppressor genes has been shown to make cancer cells radioresistant. For example the loss of the tumour suppressor gene called P16. In around half of high grade chondrosarcomas, there is damage to P16.
There may also be increased amounts of proteins in chondrosarcoma cells which prevent the cell from committing suicide or apoptosis (Ay-POP-tow-ciss) in response to the radiation. These are called anti-apoptosis proteins. Chondrosarcoma cells have been shown to have higher levels of the anti-apoptotic proteins called Bcl-2 and XIAP and Survivin.
All these research findings can suggest targets for novel and specific treatments against chondrosarcoma. For example, if the gene for Bcl-2 can be silenced or 'switched off,' the cell may respond to the radiation.
The Bone Cancer Research Trust is actively involved in funding research, especially translational research to help to find new treatments. Translational research is research that bridges the gap between promising findings in the laboratory from basic research and their clinical use in patients.
Complementary or alternative medicines (CAM)
Although complementary and alternative medicines are often called 'CAM' for short as if they mean the same thing, there are differences between them.
Other names you may see to describe CAMs are:
- traditional medicines
- unconventional medicines
- integrated healthcare/medicine
Alternative medicines or therapies, such as extract of mistletoe (iscador) and laetrile (bitter almonds), are used instead of what are called 'conventional medicines'. Conventional medicines for cancer are the treatments prescribed by doctors, for example, chemotherapy and radiotherapy.
Some people may choose to stop taking conventional medicines because they may no longer be working, or they may not wish to begin their treatment using conventional medicines for many different reasons. However, alternative medicines do not have to go through the very careful testing (trials) that conventional medicines do, and therefore may not be safe.
Adverts for alternative medicines on websites may claim to cure cancer. However, there is no scientific evidence to back these claims up. It is always best to talk to an oncologist if people are thinking about trying alternative therapies.
Complementary medicines are used alongside conventional medical treatment. Some patients use complementary medicine to help with symptoms or to aid relaxation.
Techniques used by some chondrosarcoma patients include:
- Massage therapy
- Herbal products
- Vitamins* or special diets*
- Spiritual healing.
* Patients should make sure they tell their doctors about any supplements they may be taking. Some complementary medicines, such as antioxidants, may interfere with conventional treatments.
The word 'prognosis' refers to what doctors think the chances are of the patient's cancer being cured with treatment or the likelihood of it returning. This depends on many different things, which vary between different patients.
In general, the prognosis for chondrosarcoma depends on:
- The stage of disease at the time of presentation, whether it is localised or metastatic (spread), or recurrent (come back).
- The grade of the chondrosarcoma (how abnormal the cells look).
- The type of chondrosarcoma - dedifferentiated chondrosarcoma and mesenchymal chondrosarcoma are more aggressive than conventional chondrosarcoma.
- Location of tumour, axial skeleton (skull, spine, pelvis) tumours may be more difficult to treat than tumours in the limbs.
- Adequacy of surgery.
- Whether lung (pulmonary) metastases can be removed with surgery (resectable).
Doctors cannot be absolutely certain about a patient's prognosis because each patient and each cancer can behave differently.
If you would like to see information about survival rates for chondrosarcoma please click here.
After finishing treatment, chondrosarcoma patients require follow-up care.
Outpatient hospital visits will be needed on a regular basis for the first few years after treatment, and then probably yearly after that.
These visits help the clinical team to keep an eye on a patient's general health as well as an opportunity to carry out some tests. These tests are very important because they can show up any 'late effects' from the cancer treatment. Most centres encourage patients to get in touch if they have problems between appointments.
Follow up care with an orthopaedic surgeon also helps to look out for surgery-related complications and to make sure the limb is working well.
Reaching the end of treatment can bring about mixed emotions - the thought of having no more treatment can be a cause for celebration, but this can be mixed with anxiety for the future.
The Children's Cancer and Leukaemia Group (CCLG) booklet I have finished my treatment…what happens next? is written for 10-16 year olds who have reached the end of their cancer treatment. The booklet deals with many of the emotional issues surrounding the process of returning to a 'normal' life after cancer.
Another version of this booklet has been written for the parents of a child who has had cancer My child has finished treatment…what happens next? This deals with many of the practical issues as well as emotions that might arise when a child reaches the end of their cancer treatment.
For adults who have had treatment for cancer, the issues can be more complex and include workplace and financial issues. Macmillan Cancer Care provides a wealth of useful information on living with and after cancer.
Relapse and metastases
In some patients, the cancer returns after treatment.
'Relapse' means that the cancer has returned. This can be in the same bone or area as the original cancer (local relapse) or in a different place, often in the lungs (metastasis).
If the cancer returns it will require further treatment. This treatment may involve more surgery, or possibly radiotherapy or chemotherapy. Your doctor will be able to advise you about which treatments are necessary.
What are late effects?
The surgical treatment of chondrosarcoma can involve major surgery and this can affect patients' long-term mobility. Some patients who have surgery in the pelvis may need to use a stick or crutches for walking. Patients will work together with physiotherapists and occupational therapists to improve their mobility and independence after such surgery.
'Late effects' are problems from cancer treatments such as chemotherapy or radiotherapy that may show up weeks or months after treatment has finished. Chemotherapy drugs target cancer cells but they can also damage organs and kill healthy cells. Monitoring of patients during treatment and follow up should identify problems at an early stage and therefore recognising them should limit the possibility of serious effects occurring.
|Problem/ Late Effect||Drug/ Treatment|
|Renal/ Kidney problems (nephrotoxicity)||Cisplatin|
|Heart problems (cardiotoxicity)||Doxorubicin|
|Hearing Loss (ototoxicity)||Cisplatin|
|Osteopenia (mineral loss from bone)||Radiotherapy|
|Fertility problems (more likely in males than females)||Cisplatin, radiotherapy|
|Numbness, tingling or weakness (Neurotoxicity), more likely in older adults||Cisplatin|
|Liver problems (hepatotoxicity), very rare||Cisplatin|
|Second malignancy (cancer)||Cisplatin, doxorubicin, radiotherapy when used.|
The Bone Cancer Research Trust's information has been compiled using only peer-reviewed clinical and scientific studies, reviews and case studies. Peer-review is a process in which the work of one scientist or doctor is looked at and checked by other experts in the same subject area. The peer-review process helps ensure the science is 'reliable'.
If you are interested in reading deeper into the subject, we have provided a bibliography listing the references and books we used to compile our information about chondrosarcoma.
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