Types of Primary Bone Cancer

Primary bone cancer is unusual in that it affects a wide range of age groups. It can affect children, adolescents, young adults and can also occur in older adults.

Males are at a slightly higher risk of developing most types of primary bone cancer than females

The four common forms of primary bone cancer in terms of their incidence and anatomical sites are:

• Chondrosarcoma – Chondrosarcoma is a bone cancer that develops in the cartilage cells, it is most common in adults aged between 30 and 60, and more common in the femur, tibia, pelvis, ribs and long bones.
• Osteosarcoma – is the most common form of primary bone cancer diagnosed in children and young adults.
• Ewing Sarcoma – is the second most common form of primary bone cancer in children and teenage young adults Ewing sarcoma is most common in the pelvis, chest and bones of the legs.
• Chordoma – is a rare type of cancer which arises in the base of skull or along the spine. It affects mostly adults and is often slow growing without any initial symptoms but may cause symptoms years later before being diagnosed.

Less common primary bone cancers:

• Spindle cell of the bone - Spindle cell sarcoma is a soft-tissue tumour which can start in the bone. Spindle cell sarcomas of the bone are often found in the arms, legs and pelvis. They most commonly arise in patients over the age of 40 and are extremely rare, making up just 2-5% of all primary bone cancer cases.
• Adamantinoma - Adamantinoma is a rare form of primary bone cancer that most commonly occurs in the centre of the tibia (the shinbone) and can also affect the fibula (the calf bone). Adamantinomas can develop at any age, but most common in young adults (20-35 years).
• Angio sarcoma of the bone - Angiosarcomas are aggressive yet very rare cancerous tumours that present in any area of the body - usually in the soft tissues. They are most reported in the breasts, heart, skin, liver and bones.They can present at any age but are most common in patients between 20 to 50 years of age.
• Giant cell tumour (non-cancerous) - Giant cell tumours of the bone tend to be benign, but they can be locally aggressive. The majority of all giant cell tumours of the bone occur in patients aged between 20 and 45 years of age.
• Ameloblastoma - Ameloblastoma is a rare, benign, tumour of the bone which can occur in the lower or upper jawbone. Although it can occur at any age, ameloblastoma it is most frequently seen in patients of around 30 to 40 years of age.

If you have symptoms of a primary bone cancer you should visit your GP who will be able to make a referral to a specialist or for further investigations such as x-ray and blood tests.If the x-ray suggests a possible primary bone cancer your GP should refer you to a bone cancer centre.

Symptoms of primary bone cancer can vary depending on the size and location of the tumour. Symptoms can be general and appear like other common problems such as sports injuries, tendinitis. arthritis and growing pains. As primary bone cancers are rare, and some GP’s will never come across a case in their career they are often misdiagnosed. For more information on the symptoms of bone cancer, see here.

If a primary bone cancer or bone tumour is suspected, you will be referred to a to a bone cancer centre, where several investigations will take place which may include:

X-ray

An X-ray is a quick and painless procedure commonly used to take images of the inside of the body. X-rays are effective at looking at abnormalities in the bone and can help detect many conditions. For more information on X-rays, please click here.

CT (Computerised Tomography) Scan.

A CT scan is a machine that uses X-rays to create detailed 3D images of the inside of a body. The CT scan helps the doctors understand better the size and location of the tumour and if it has spread to other parts of the body. Sometimes a dye known as an IV contrast is administered whilst you have the CT scan which helps highlight areas of concern and to see structures inside the body more clearly.

For more information on CT scans, please click here.

MRI (Magnetic Resonance Imaging).

An MRI scan uses magnetic fields and radio waves to produce detailed images of the inside of the body.

Just like a CT scan, IV contrast can be used to achieve more detail.

The information gathered will be used to stage the tumour and plan what course of treatment is best for you.

For more information on MRI scans, please click here.

PET (Positron Emission Tomography)

A PET scan is used to produce 3D images of the whole body.

A harmless radioactive substance known as a radiotracer is injected into your arm. Cancer cells take up the radiotracer more rapidly than normal tissue, and a special camera then creates a picture of areas of radioactivity in the body. The picture is not detailed like a CT or MRI scan, but it provides useful information about the whole body.

PET scans can help show the spread of bone cancer to the lungs, other bones, or other parts of the body. They can also be used to see how well the cancer is responding to treatment.

For more information on PET scan click here

PET scans are often combined with CT and MRI (PET- MRI) scans (PET-CT) to create a more detailed picture.

Isotope Bone Scan

An isotope bone scan is a method of looking at your bones to show conditions not seen using X-rays. It requires the injection of a small amount of radioactive fluid, known as a radionuclide which is taken up by the bones and detected by a gamma camera

Areas where the radionuclide is taken up are called a “hot spots' and appear as dark spots on the image. Hot spots can represent where cancer cells may be present or other medical conditions such as arthritis.

For more information on isotope bone scans click here.

Blood tests

Blood tests will be taken regularly for diagnosing your cancer and throughout treatment.

The results of a blood test can highlight areas of concern – for example if you have an infection.

For more details on Blood Test please click here.

Bone biopsy

Tumours found in the bone may be benign (non-cancerous) or malignant (cancerous). To find out whether the tumour is benign or malignant, a biopsy sample must be taken and assessed.

A small sample of the tumour is taken and examined under a microscope by a pathologist. This helps doctors establish the type of cancer, as well as the stage and grade of the primary bone cancer. This information is important as it will determine what course of treatment will be taken. There are 2 main types of biopsies – needle biopsy and open biopsy.

Some patients with an Ewing sarcoma may undergo a bone marrow biopsy to determine whether the Ewing sarcoma has spread to the bone marrow.

For more information on bone biopsies, click here.

These diagnostic tests and investigations help your doctor to work out:

• The grade of the cancer- how the cancer looks under a microscope which can be used to predict how the cancer might grow and spread
• The stage of the cancer - how big it is and whether it has spread outside the immediate vicinity of the tumour Identifying the stage and grade of the cancer enables your doctor to advise on the best course of treatment for you.

The grading and staging of primary bone cancer can seem complicated. There are 2 main systems for staging your primary bone cancer and the grading is slightly different for each system.

Grading Primary Bone Cancer

Doctors grade cancer cells according to how the cells look under a microscope. The grade of the bone cancer gives your specialist a guide to how the cancer may behave. The most common grading system for bone cancer uses two grades:

• Low grade cancers have cells that look slightly abnormal. These cancers usually grow slowly and are less likely to spread

• High grade cancers have cells that look very abnormal. These cancers are likely to grow more quickly and are more likely to spread.

Staging Primary Bone Cancer

The stage of a cancer tells your doctor how big it is and whether it has spread.

Your doctors and surgeons might use one of 2 different systems to stage your bone cancer. These are:

• the Enneking stages (surgical)
• the TNM stages

They should explain which one they are using and what this means for you.

The Enneking staging system

The Enneking staging system describes size and grade of the tumour and if it has spread. It helps your surgeon decide how much bone to remove during surgery.

TNM staging system

TNM stands for Tumour, Node and Metastases:

In the TNM system, bone cancer cells are graded from GX to G4:

• GX means the grade cannot be assessed
• Grades 1 and 2 are low grade tumours
• Grades 3 and 4 are high grade tumours

For more detail on each stage of primary bone cancer, please click here.

The Nice Guidelines for Improving Outcomes for People with Sarcoma recommends that anyone with a primary bone cancer should be referred to a bone cancer centre for diagnosis and treatment. Some benign tumours such as giant cell tumours are also diagnosed and treated at bone cancer centres. NICE Guidelines.

MDT (Multi-Disciplinary Team)

An MDT consists of a group of healthcare professionals who will decide on what course of treatment is best for you and will be involved at different stages of your treatment. Below is a list of specialists and their roles during your treatment pathway:

The MDT includes:

• Specialist bone cancer surgeons
• Specialist oncologists - doctors, nurses and radiographers who treat cancer with chemotherapy and/or radiotherapy
• Histopathologists - specialist that studies tissues taken from biopsies or surgery under a microscope which helps grade the tumour
• Radiologists – Specialist who assess a cancer by looking at images produced by X-rays or other scans
• Clinical Nurse Specialist (CNS) or Advanced nurse practitioner. They have an in-depth knowledge of bone cancer, treatment and management. They also act as a point of contact and support for patients and their families.

There are 5 MDTs specialising in bone cancer treatment and surgery planning in England. These are based at the 5 bone cancer centres where most bone sarcoma investigations and surgeries should take place. See Bone Cancer Centres for a list of these hospitals.

Surgery

In most cases patients with a primary bone cancer are required to have surgery. This will be carried out at one of the 5 bone cancer centres.

The decision for surgery to be performed will be made by the MDT and carried out by an experienced surgeon The extent of the surgery will depend on the location, stage and grade of the tumour. The aim of the primary bone cancer/ bone tumour surgery is to completely remove the primary tumour with as little effect to the body as possible so that the tumour can no longer grow or spread.

Different surgical techniques used are:

• Surgical resection

If possible, only the tumour will be removed with little or no effect to the body’s function and no need for the bone to be rebuilt.

• Autograft

The affected bone is removed and replaced with a piece of healthy bone from elsewhere in the body.

• Allograft

Patients have their tumour removed and the affected area of the limb is rebuilt using donated tissue.

• Irradiation/Re-Implantation

Diseased bone is removed, treated with radiation to kill any tumour cells, and then put back into the patient.

• Metal Implant

The affected bone is replaced with a metal endoprosthetic replacement EPR (known as an endoprosthesis).It is custom made for the individual patient. If the tumour is near a joint, the joint can also be replaced. This is a very common technique for primary bone cancers of the arms, legs, shoulder or pelvis.

• Amputation

An amputation is the surgical removal of part of the body such as an arm or leg. An amputation for primary bone cancer is carried out if the tumour has spread to major blood vessels, nerves in the affected bone or the patient has suffered a severe infection after surgery. To find out more information, please refer to our amputation videos here

If your cancer has spread e.g., to the lungs, you may require further surgery. If surgery is not possible you may have chemotherapy or radiotherapy instead.

Surgery for PBC is very difficult, you will have support from physiotherapy, occupational therapy and rehabilitation to help you improve movement and get used to a prosthetic.

Chemotherapy

Chemotherapy is a cancer treatment where drugs are used to kill cancer cells.They work throughout the body and are called a systemic treatment. You may receive this at one of the bone cancers centres or one of the sarcoma specialist hospitals.

Paediatrics will have their chemotherapy at principal treatment centres. Chemotherapy may be given before surgery (referred to as neo-adjuvant chemotherapy) or after surgery (referred to as adjuvant chemotherapy), depending on the specific needs of the patient.

• Neoadjuvant Chemotherapy: aims to shrink the tumour and kill any cancer cells that have spread to other areas of the body before the patient has surgery. This creates an easier surgical procedure and allows a more sparing removal of the affected area of the tumour.
• Adjuvant Chemotherapy: is given after surgery and kills any remaining cancer cells in the body. This method ensures the tumour is less likely to return later and greatly improves the cure rate for primary bone cancers.

most chemotherapy is delivered in ‘cycles’ which include rest periods to help your body recover from side effects.

To find out more about chemotherapy and the side effects here.

Chemotherapy may be given as part of a clinical trial. A clinical trial is a study used to investigate new treatment options or side-effects of treatments. The MDT at the bone cancer centre or specialist sarcoma centre will let patients know about any clinical trials that are available.For more information on clinical trials please visit our Clinical Trials section of this website.

Stem Cell Transplants

A stem cell transplant or bone marrow transplant replaces damaged blood cells with heathy ones.

Stem cells are special cells made in the bone marrow (a spongy tissue found In the centre of bones) that can turn into different types of blood cells (reference nhs.co.uk)

Red blood cells which carry oxygen around the body,

White blood cells to help fight infection and

Platelets – which help to stop bleeding.

Find out more about Stem Cell Transplants here

Radiotherapy

Radiotherapy is a localised treatment using high energy x-rays to destroy cancer cells.

Radiotherapy can be given before surgery to shrink the tumour, after surgery to remove any remaining cells or as a standalone treatment for patients who have an inoperable tumour (for example in the spine).Radiotherapy can also be given palliatively to control symptoms such as pain.

Proton Beam Therapy

Proton Beam Therapy a type of radiotherapy that uses high energy protons instead of high energy x-rays. Protons can deliver high energy to the target whilst reducing side effects which is why proton beam therapy is suitable for treating tumours close to vital or delicate organs. Proton beam therapy is available at two centres in the UK: Manchester Christie Hospital and University College London Hospital.

Find out more about PBT here

Receiving a cancer diagnosis is a very anxious time for patients and their families. Often you are inundated with complex information, which can make it difficult to think straight. To make things easier, we have created a list of questions that you may want to ask your medical team.

• What treatment will I receive and why has this treatment plan been decided?
• Are there any likely side-effects to this treatment?
• Will I need medication to control these side-effects?
• How long will my treatment last?
• Where will my treatment take place?
• Will this treatment affect my day-to-day life, such as my ability to attend work/school?
• Will I need follow-up tests to ensure the treatment has worked?
• Am I at risk of any effects later in my life, and if so, is there anything I can do to prevent these before my treatment begins? (For example, some types of cancer treatment can affect a patient's fertility, so this is something that is worth considering before starting your treatment)
• Will I need rehabilitation treatment, and if so, how will it be arranged and where will it take place?
• Are there any clinical trials available that I could take part in?

Rehabilitation

Following treatment, many patients benefit from rehabilitation services. Rehabilitation is a form of therapy that enables patients to regain strength, tackle day-to-day activities and return to normal life as quickly as possible following treatment. This process of therapy is required following many conditions to enable the patient to have the best quality-of-life possible and may begin as little as 24 hours after surgery.

If you are a patient who requires any of these services during your treatment, or further support following your treatment, please contact a member of your medical team to request a referral to a rehabilitation service.

At the start of their rehabilitation patients will be given advice, and possibly some exercises to carry out to improve strength, fitness and the ability to move around independently or in a wheelchair.

The main rehabilitation services include:

• Physiotherapists: help patients return back to an active lifestyle as quickly as possible to restore strength, movement and function
• Occupational Therapists: help patients to complete day-to-day activities in order to regain their independence
• Dieticians: advise on the most appropriate nutrition for patients during and after their treatment
• Psychological Support and Services: psychologists will support patients through any feelings of anxiety or depression to overcome the concerns that often come with a cancer diagnosis
• Local Support Groups: many support groups are organised and run locally. It is best to ask your clinical nurse specialist for information on these local services. The Bone Cancer Research Trust offers a Support & Information Service for all primary bone cancer patients and their families. For more information on our groups and digital support groups, click here. We also run a Freephone Support Line (Mon-Fri 9-5pm) on 0800 111 4855 or you can drop us an email. If your clinical nurse specialist is a long way from where you live, you may find that your local District Nurses or District Children’s Nurses may be more aware of what is available locally.
• Prosthetists: specialists who design and create prostheses (artificial limbs) following amputations to match as closely as possible to the individual patient’s removed limb
• Orthotists: specialists who provide aids for patients following surgery, such as splints, special footwear or other supports

Follow-up

After your treatment has finished, most patients will have regular follow-up appointments. At these appointments you will be examined, asked about any symptoms or side effects you may be having and if you have any concerns. You may also have blood tests, chest X-rays, MRI and CT scans.

These appointments help your doctor or CNS to identify any possible problems early on. How often you have follow-up appointments depends on the type and stage of your cancer, the types of treatment you have had and your individual needs. It also depends on the arrangements of your hospital.

Late Effects

Most patients experience side effects during their treatments which go away or improve after treatment is ended. However, some side effects become permanent or develop months or years after treatment has ended. These are known as late effects of cancer treatment. More is becoming known about late effects as people are living longer after being treated for cancer.

The late effects of cancer treatment vary depending on the type of cancer, the treatment and surgeries and the age of the patient when undergoing treatment.

However, not everyone who has cancer treatment will necessarily experience late effects. Different chemotherapy drugs cause different late effects and late effects of radiotherapy and surgery will affect only the area of the body exposed to them.

If you were treated for cancer as a child, you may be at risk of the same late effects as people who were adults during their cancer treatments and additional late effects as children's tissues, organs and bones grow rapidly and cancer treatment can interfere with this critical time of growth.

As with late effects in adults, late effects from childhood cancer treatment will vary depending on the type of cancer and type of treatment. Additionally, the age at which you were treated may determine which late effects you might experience.

Signs and Symptoms of Late Effects

You can talk to your doctor or CNS about the late effects of your particular treatment or surgery. They should be able to advise you on which late effects you are at risk from. However, the late effects of many cancer treatments still often go undiagnosed.

At your follow-up appointments you will be screened for late effects of your cancer treatment and surgery. It is also an opportunity to talk to your doctor about any signs or symptoms you may be suffering from and to discuss whether these are late effects.

If you were treated for cancer many years ago or are no longer having follow-up appointments, you should talk to your GP about late effects or contact a Late Effect Clinic.

Late Effects Clinics

If you had your cancer treatment as a child you will be monitored for late effects by your oncology team or a late effects clinic specifically for those patients treated as children. For all other cancer patients, they will be screened by their oncologist whilst under their care but are no longer monitored once they are discharged by their oncology/surgical team.

In September 2014, a late effects clinic was established at Nottingham City Hospital.This Late Effects Clinic is a bespoke service which aims to support people suffering with long-term effects from radiotherapy and chemotherapy and offers practical advice and signposting for a range of physical and psychological effects. It offers a wide range of support and guidance regarding late effects such as bowel and bladder problems, infertility, osteoporosis, sexual dysfunction, pain management and psychological issues to name a few. Patients self-refer and it is open to all patients who are a minimum of 6 months out of treatment. There is no upper limit on time from finishing treatment.

Since this clinic was established, similar clinics have been set up in Derby, Sheffield and Taunton and many more are developing throughout the country.

There is also a Complex Cancer Late Effects Rehabilitation Service based at The Royal National Hospital for Rheumatic Disease in Bath which is a two-week inpatient rehabilitation service for those patients with complex, chronic late effects. Referral to this service is via the late effect clinic or local pain services.

You may find our webinar on the Late Effects Clinic with radiographer, Emma Hallam helpful.

Key References:

1. WHO Classification of Tumours Editorial Board. 2020. Soft Tissue and Bone Tumours. Fifth Edition.

2. Gerrand, C, Athanasou, N, Brennan, B, Grimer, R, Judson, I, Morland, B, Peake, D, Seddon, B, and Whelan, J. UK Guidelines for the Management of Bone Sarcomas. Clinical Sarcoma Research. 2016; 6(7). Available at: https://clinicalsarcomaresearch.biomedcentral.com/...

3. National Institute for Health and Clinical Excellence (NICE). Referral guidelines for suspected cancer: recognition and referral (June 2015).

4. National Institute for Health and Clinical Excellence (NICE). Improving Outcomes for People with Sarcoma: The Manual. (March 2006).

5. National Cancer Intelligence Network. 2010. Bone Incidence and Survival Rate in England. Find it here.

6. National Cancer Intelligence Network. 2012. Bone Incidence and Survival Rate in England. Find it here.

7. NHS England. Proton Beam Therapy. 2020. Find it here.

Further Reading:

Barr RD, Wunder JS ‘Bone and soft tissue sarcomas are often curable -- but at what cost?’ Cancer. 2009; 115(18): 4046-4054. Available at: http://onlinelibrary.wiley.com/doi/10.1002/cncr.24...

Blunt M. Chemotherapy, Cakes and Cancer (An A to Z survival guide for living with childhood cancer). Published by CLIC Sargent, www.clicsargent.org.uk.

Clarke, R.K, Anatomy and Physiology: Understanding the Human Body, 2005. Jones and Bartlett Publishers Inc, Massachusetts, ISBN 0-7637-4816-1 Available here: https://books.google.co.uk/books?id=4idMNpe04qgC&p...

DiCaprio MR, Friedlaender GE. Malignant Bone Tumors: Limb Sparing Versus Amputation. J Am Acad Orthop Surg. 2003; 11(1): 25-37. Available at: http://www.ncbi.nlm.nih.gov/pubmed/12699369

Eggly S, Harper F, Penner L, Gleason M, Foster T, and Albrecht, T; Variation in Question Asking during Cancer Clinical Interactions: a Potential Source of Disparities in Access to Information. Patient Education and Counselling. 2011; 82(1):63-68. Available at: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC294353...

Eroschenko, VP, diFiore’s Atlas of Histology with Functional Corrections, Eleventh Edition, 2008. Lippincott Williams and Wilkins, Baltimore. ISBN-13: 978-0-7817-7057-6. Available at: https://books.google.co.uk/books?id=JZoTjdNSlpQC&p...

Heare T, Hensley MA, Dell'Orfano S. Bone tumors: osteosarcoma and Ewing's sarcoma. Curr Opin Pediatr. 2009; 21(3): 365-72. Available at: http://www.ncbi.nlm.nih.gov/pubmed/19421061

Kleihues P, Sobin L, Fletcher C et al, (eds.) WHO Classification of Tumours: Pathology and Genetics of Tumours of Soft Tissue and Bone, Lyon, France: IARC Press Available at: https://www.iarc.fr/en/publications/pdfs-online/pa...

Langer T, Stöhr W, Bielack S, Paulussen M, Treuner J, Beck JD; German Late Effects Working Group in the German Society of Pediatric Oncology and Hematology. Late effects surveillance system for sarcoma patients. Pediatr Blood Cancer. 2004; 42(4): 373-9.