How is chondrosarcoma treated?



This information has been written for patients, their families and friends, and the general public to help them understand more about chondrosarcoma: what it is and the different types. This information is produced in accordance with BCRT's information policy.

The main treatment for chondrosarcoma is surgery to remove the tumour.

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:

  1. What kind of cancer is this?
  2. 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.

1) 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.

2) 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 show 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.

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.

(Onishi 2011, Chow, Novais and Lor Randal 2010, Riedel et al., 2009, Lor Randall and Hunt, 2010, Gelderblom et al., 2008).

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.

(Riedel et al., 2009, Novais and Lor Randal 2010, Gelderblom 2008, Leerapun et al., 2007, Veth et al., 2005)

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 is to, where possible, 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.

(Riedel et al., 2009, Novais and Lor Randal 2010, Gelderblom 2008, Leerapun et al., 2007, Veth et al., 2005)

Tumour removal can be very complicated, and requires very careful individual planning for each patient. For example 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

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 (me-ZEN-ky-mal) 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

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. For information on how chemotherapy works, please click here

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

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 2017. 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.

For more information about how this treatment can be accessed by NHS patients, click here for the NHS Specialist Services website about proton beam therapy.

(Onishi 2011, Ares et al., 2009, Riedel et al., 2009, Frank and Selek, 2010).

Where does treatment for chondrosarcoma 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

For patients in England, diagnosis and surgery should take place in one of the Bone Cancer Centres (see map below).

For young patients treatment should be provided at one of the UK Specialist Children’s Cancer and Leukaemia Cancer Centre, Young Adult Cancer Centre, or Teenage Cancer Trust Unit.

Scotland

In Scotland the 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 go to Glasgow, Edinburgh or Aberdeen.

Republic of Ireland

Most patients in the Republic of Ireland aged under 16 receiving chemotherapy for primary bone cancer go to Our Lady’s Hospital, Crumlin, Dublin.

Patients aged 15-19 are seen at Mater Misercordiae Hospital, Our Lady’s Hospital, Crumlin and Waterford Regional Hospital.

Patients aged over 20, are seen 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.

Most patients (all ages) will go to St. Marys Orthopaedic, Cappagh for surgery, and most patients (all ages) will attend St Luke's and St Anne's Hospital, Dublin for radiotherapy. However, some patients may also attend other hospitals in Dublin and Cork.

Figure 4. Bone Cancer Centres and Children and Young Adult Treatment Centres in the UK and Ireland.

Figure 4.  Bone Cancer Centres and Children and Young Adult Treatment Centres in the UK and Ireland.

Key

Red starsSpecialist Children’s Cancer and Leukaemia Centre
Blue starsBone Cancer Centre
Green starsChildren and Young People’s Integrated Cancer Service
Purple starsTeenage Cancer Trust Unit
Orange starsScottish Sarcoma Network Hospital

Bone Cancer Centres:

Most surgery for primary bone cancers in the UK is carried out at one the Bone Cancer Centres. These should also be the place where diagnostic tests, including bone biopsies, are carried out.

  • 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

Children's and Young Adults Specialist Cancer Treatment Centres:

For children, teenagers and young adults chemotherapy and radiotherapy are usually given at a Children’s and Young Adults Specialist Cancer Treatment Centre. If this is a long way from the patient’s home then a “shared care” arrangement can be made with a local hospital. This means that the MDT at the Children’s Centre designs a treatment plan, and the local hospital administers it.

  • The Royal Liverpool and Broadgreen University Hospitals NHS Trust
  • Children’s Hospital for Wales, Cardiff
  • Great Ormond Street Hospital, London
    • Shared care from here is offered at:
             St Georges Hospital
             Queen Elizabeth the Queen Mother Hospital
             Royal Alexandra Children’s Hospital, Brighton
             William Harvey Hospital
             Buckland Hospital Dover
             Kent and Canterbury Hospital
             Medway Maritime Hospital
  • Great North Children’s Hospital, Royal Victoria Infirmary, Newcastle Upon Tyne
  • Royal Belfast Hospital for Sick Children
  • Sheffield Children’s Hospital
  • Leeds General Infirmary
  • Our Lady’s Children’s Hospital, Dublin
  • Birmingham Children’s Hospital
  • Western Park Hospital, Sheffield
  • Beatson West of Scotland Cancer Centre, Glasgow
  • University College Hospital London
    • Shared care from here is offered at:
             Royal London Hospital
             Meadway Maritime Hospital
             Royal Alexandra Childen’s Hospital, Brighton
  • Bristol Royal Hospital for Children
    • Shared care from here is offered at:
             Great Western Hospital, Swindon
             Gloucester Royal Hospital
             Derriford Hospital Plymouth
             Royal United Hospital Bath
             Musgrove Park Hospital Taunton
             Yeovil District Hospital
  • Alder Hey NHS Foundation Trust
    • Shared care from here is offered at:
             Wrexham Maelor Hospital
             Ysbyty Glan Clwyd
  • Addenbrooks Hospital Cambridge
    • Shared care from here is offered at:
             Ipswich Hospital
  • The Royal Marsden Hospital
    • Shared care from here is offered at:
             St Georges Hospital
             Royal Alexandra Children’s Hospital, Brighton
             East Surrey Hospital, Redhill
  • East Midlands Children’ and Young People’s Integrated Cancer Service
    • Formed from:
             Leicester Royal Infirmary
             Queen’s Medical Centre, Nottingham
  • Queen Elizabeth Hospital, Birmingham
  • Yorkhill Royal Hospital for Sick Children, Glasgow
    • Share care from here is offered at:
             Raigmore Hospital, Inverness
  • Royal Manchester Children’s Hospital
  • Southampton General
    • Shared care from here is offered at:
             Royal Alexandra Children’s Hospital

For older teenagers and young adults there are also specialist centres. These are sometimes part of a usual hospital ward, or some are designed and built by the Teenage Cancer Trust.

Teenage Cancer Trust Units:

  • Birmingham
    • Queen Elizabeth Hospital Young Person Unit
    • Birmingham Children’s Hospital
    • Royal Orthopaedic Hospital
  • Addenbrooke’s Hospital Cambridge
  • University Hospital of Wales, Cardiff
  • Royal Hospital for Sick Children, Edinburgh
  • Glasgow:
    • Beatson West of Scotland Cancer Centre
    • Royal Hospital for Sick Children (Yorkhill)
  • Castle Hill Hospital, Hull
  • Leeds:
    • St James’s University Hospital Young Adult Unit
    • Leeds General Infirmary
  • Liverpool:
    • Alder Hey Children’s Hospital
  • London:
    • University College Hospital
    • University College Hospital Cancer Centre
  • Manchester:
    • The Christie Hospital Cancer Centre
  • Newcastle:
    • The Great North Children’s Hospital
    • The Freeman Hospital
  • Republic of Ireland
    • Our Lady’s Hospital, Crumlin (under construction)
  • Sheffield:
    • Weston Park Hospital
    • Royal Hallamshire Hospital
  • Southampton General Hospital
  • Surrey:
    • The Royal Marsden Hospital
  • The Wirral
    • Clatterbridge Centre for Oncology

Other teenage and young adult specialist centres:

  • Leicester Royal Infirmary – Young Adult Oncology Service (CLIC Sargent)
  • Sheffield Teaching Hospital NHS Foundation Trust
    • Care for 13-16 year olds at Sheffield Children’s hospital
    • Care for 16-19 year olds at Weston Park Hospital or Royal Hallamshire Hospital
    • Care for patients aged 19-25 can choose which hospital to be treated at.
Who is involved in deciding the treatment for chondrosarcoma?

Patients in the UK and Ireland are looked after by a team of specialists, called a ‘Multidisciplinary Team’ (MDT). The Multidisciplinary Team will analyse the test results to reach a diagnosis.

The MDT for treatment will consist of:

  • Specialist bone sarcoma surgeons

  • Specialist sarcoma oncologists (oncologists are doctors who look after people with cancer)

  • Specialist sarcoma pathologist (pathologists are doctors who use laboratory techniques to diagnose disease)

  • Radiologists (doctors who diagnose disease and conditions from looking at x-rays, or scans)

  • Cytogeneticists (scientists who specialise in detecting damage to genes that help control the cell)

  • Sarcoma cancer nursing specialists (sometimes called ‘CNS’) perform an essential role in treating and caring for bone cancer patients. These nurses are specially trained to look after sarcoma patients.

  • Physiotherapists and occupational therapists will help with rehabilitation (rehab) after surgery.

  • Dieticians

  • Social workers and psychologists will help with patients’ emotional, social and educational needs.

There is more about patients’ experiences in our My Bone Cancer Story section.

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.

The importance of clinical trials

If standard therapies are not effective then a patient may be offered the opportunity to go onto a clinical trial. Doctors use clinical trials to test new treatments or changes to existing ones. Clinical trials usually have three stages called phases:

Phase 1 Clinical Trial: If a drug looks promising in laboratory studies, a phase 1 trial may be carried out. This is usually the first time a new drug is tried in people. These types of trials do not usually look at the effect on specific types of cancer but look at things such as side effects and the safest and most effective dose. These types of trials are usually done with a small number of people, usually 10-30.

Phase 2 Clinical Trial: This type of trial may be open to people with specific types of cancer or a number of different cancers. They are usually carried out on more patients than phase I trials, usually around 100; mainly to look at which type of cancer the drug works best against, to look at the best dose and side effects again, and to find out if the drug is worth taking to a much larger phase 3 clinical trial.

Phase 3 Clinical Trial: If a drug in a phase 2 trial looks as if it works as well or better than an existing treatment, a phase 3 trial is carried out. These trials are usually open to people with a specific type of cancer. Many more people are included in these trials, this helps to make sure doctors can see how well the drug may or may not help.

The new drug or treatment is usually compared to an existing treatment. Patients will be put into one of at least two groups, for example, either the 'new drug group' or the 'existing treatment group'. This is done randomly by a computer. Randomisation reduces bias and also helps doctors to be certain that it was the drug that caused the effect and it did not just happen by chance.

You may see the terms 'blind' or 'double blind' randomised clinical trial. A blind trial means the patient does not know if they are receiving the existing treatment or the new one. In a double blind trial neither the doctors nor the patients know which treatment they have received.

A phase 3 clinical trial can be carried out in hospitals in many countries at the same time, in order to reach more patients.

Clinical trials may not always be available, as the patient may not meet some of the requirements to be treated on a trial. If a clinical trial is suggested by the doctor, they will speak to the patient and give them information to read about the trial.

Doctors will describe what taking part in the trial will mean for the patient, what happens before and during the trial and about possible risks. Patients will be given time to think about whether they wish to take part in the trial.

If a clinical trial is suggested by a doctor patients or parents may want to think about the benefits and risks involved in taking part in a clinical trial before they make a decision. Benefits may be that parents and patients will have an active role in his or her health care, may have access to new treatments before they become generally available, and will help others by contributing to medical research. Patients who enrol in clinical trials are carefully monitored and so their health may benefit just from taking part in the trial.

Risks could include side effects of the drug, which may be unpleasant, or the treatment may not be safe or effective for the patient. The trial may involve frequent stays in hospital and extra cost. It may be helpful to have a list of questions written down when you meet the trial co-ordinator.

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.

(Kim et al., 2011, David et al., 2011, Chow 2007, Riedel et al., 2009, Novais and Lor Randall, 2010 Johnson et al., 2010).

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.

(Lechler et al., 2011, Kim et al., 2011, Onishi et al., 2011, David et al., 2011, Kim et al., 2007, Kim et al., 2009, Moussavi-Harami et al., 2006, Asp et al., 2000. Bedikian et al., 2006, Wiedenmann et al., 2007)

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 Medicine (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' and '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:

  • Acupuncture
  • Massage therapy
  • Herbal products
  • Vitamins* or special diets*
  • Visualization
  • Meditation
  • Spiritual healing.

(National Cancer Institute, 2010)

* 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 authors and reviewers of this information are committed to producing reliable, accurate and up to date content reflecting the best available research evidence, and best clinical practice. We aim to provide unbiased information free from any commercial conflicts of interest. This article is for information only and should not be used for the diagnosis or treatment of medical conditions. BCRT can answer questions about primary bone cancers, including treatments and research but we are unable to offer specific advice about individual patients. If you are worried about any symptoms please consult your doctor.

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Version 2 produced January 2013
Information will be reviewed in January 2015

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