The following are brief notes on Primary Bone Cancer.
BONE CANCER (Bone Sarcoma).
There are more than 200 bones in the human body. Bones support and protect the body, as well as allowing us to move.
There are 2 types of cells in the bones, called osteoblasts and osteoclasts. The osteoblasts build up the bone framework, while the osteoclasts break it down. Between them they keep the bones healthy.
Primary bone cancer (PBC).
Primary bone cancer is cancer that starts in the cells of the bone, rather than starting in another part of the body. It then spreads to the bone.
Secondary bone cancer.
This is where cancer has spread from another part of the body into the bones. For example, it may have started in the breast, kidneys, lungs, prostate or thyroid. These forms of cancer usually affect older people.
More about PBCs.
Primary bone cancer (PBC) is a very rare cancer and is amongst the 53% of all cancers that are classified as rare. There some 550 cases of PBC in the UK every year. The cancer is medically known as a sarcoma or bone tumour and is an abnormal growth of cells within the bone that may be noncancerous (benign) or cancerous (malignant).
There are 4 main PBCs:-
· Osteosarcoma (osteogenic sarcoma)
· Ewing’s sarcoma
· Spindle Cell Sarcoma
The cause of PBC is unknown but there are thought to be some risk factors including:-
· Exposure to radiation
· Treatment with some chemotherapy drugs
· Certain bone diseases
· Rare inherited genetic conditions
Often people think that a knock or injury to a bone can cause cancer. It seems more likely that an injury shows up a cancer that is already there or a bone affected by cancer may be weakened and so is more likely to be damaged in an accident. This is one of the key areas for which more research is needed.
Osteosarcoma and Ewing’s sarcoma – the teenage and young adult cancers.
Every day, 6 young people will be told that they have some kind of cancer. 2 of those 6 will be told that it is a primary bone cancer – meaning that although PBC is a rare cancer, for young people it is the predominant cancer, with chances of survival beyond 5 years being around 55% - a figure that in the UK has not changed in 25 years. Some 90% of all new cases of PBC will be teenagers or young adults.
Because both of these sarcomas predominantly affect an age group undergoing a period of rapid growth – the teenage years – there are thoughts that the development of the bones – particularly the long bones - is, in some way, affected by the growing process.
For both osteosarcoma and Ewing’s sarcoma, there will be more boy patients than girls.
Osteosarcoma is the most common type of PBC. There are a few childhood cases but the majority are in teenagers and young adults (up to 24 years old). Cases of osteosarcoma appear to peak at around 17 years old. The bones mostly affected are:-
· Shin (near the knee)
· Thigh (near the knee)
· Upper arm (near the shoulder)
However, osteosarcoma can occur in any bone.
Ewing’s sarcoma is the second most common PBC. The main age range is slightly different - 10 to 20 years – although 10% are over 20 years old. Cases of Ewing’s sarcoma appear to peak at around 15 years old. The bones mostly affected are:-
Ewing’s sarcoma tumours can also develop in the soft tissues.
This is where pieces of the cancer have, in effect, broken off and have entered other sites in the body. The most common is invasion of the lungs (pulmonary metastasis).
· Bone fracture subsequent to a minor incident
· Limited movement if cancer occurs near the joint
· Bone pain - which may be worse at night
· Tenderness, swelling or redness at the site of the tumour
It is likely that the patient’s General Practitioner will be the first person to be consulted. It should be remembered that, because PBC is so rare, the average GP will go through their career without seeing a bone cancer case. For this reason there is a risk of early stage misdiagnosis. Teenagers are going through a growing spurt and may be keen on sport. These 2 factors can cause a GP to think that the “pain” is, in fact, “Growing Pains” or the result of a “Sports Injury”. However, if the patient shows no signs of recovery, referral to a hospital should be requested. Once there, a range of tests may be used to gather detailed information including:-
· A plain X-Ray
· Bone scans – CT (Computerised Tomography) and MRI (Magnetic Resonance Imaging)
· Blood tests
Early diagnosis is considered to be crucial in improving the chances of long term survival so, if any of the above symptoms – or other unusual symptoms – are experience, then do not delay in seeking medical advice.
Before treatment starts, a number of events may occur or discussions take place about what will happen during treatment.
Fitting a Hickman Line or Portacath.
During treatment it will frequently be necessary to take blood samples as well as administering chemotherapy drugs and other solutions. Making a fresh injection every time this is required would be impractical as well as uncomfortable. So Patients’ may be fitted with either a Hickman Line or Portacath, which means it will be there all the time – including time spent at home during the course of treatment. These are both very practical and help patients to feel more comfortable.
The Hickman Line is inserted into a vein usually adjacent to the shoulder, with a second line on the chest wall. The Line is then threaded towards the heart. The other end is taped to the patient’s body with the socket which will receive the syringe left exposed. Extreme care needs to be taken to see that this socket is kept clean, as catching an infection can elongate the treatment period.
The Portacath is inserted under the skin, usually towards the side of the abdomen or the chest. When healed, it looks rather like a lump under the skin. When samples of blood are taken or when drugs are administered, a special needle know as a Huber Needle is placed over the lump.
More information can be obtained on the following NHS sites.
Chemotherapy treatment may lead to hair loss. The most visible sign is hair loss to the scalp (Alopecia) , but generally hair from all over the body will disappear including eyebrows. It will grow back but may be different in texture and colour. Wearing a wig or bandana during treatment is popular
An informative National Cancer Institute site is:-
Details on wigs can be found on www.mynewhair.org. My New Hair is a Registered Charity.
Infertility and Assisted Conception.
Chemotherapy may affect both men and women, making them infertile. Talk to your Doctor before treatment. Referral to a fertility specialist may be recommended, as well as to the Assisted Conception Unit at a hospital or clinic. It may be possible for ova or sperm to be stored before treatment starts.
Two National Cancer Institute sites for men and women separately are:-
On Assisted Conception the Infertility Guidance Service site is:-
Treatment may affect some body organs, so patients may be told that throughout their period of treatment, they will undergo checks on their heart and other organs.
Preparation for a reduced immune system.
The body’s immune system is likely to experience a reduction in effectiveness. To lessen the risk, the patient, family and visitors may be advised to be particularly careful about hygiene when in hospital and at home :-
· Cleanliness – hand and body washing with anti-bacterial solutions; sterilizing of food preparation areas; toilet cleaning.
· Boiling all drinking water and water used in cooking. Time needs to be allowed for this to cool after boiling and it needs to be replaced regularly.
· Risk of infection. Visits by people who themselves are suffering from an infection should be actively discouraged.
A good website for general information is http://www.patient.co.uk/health/Chemotherapy.htm
Osteosarcoma will usually be treated with a course of high-dose chemotherapy to reduce the size of the tumour, prior to surgery. Often the chemotherapy will kill off any other small areas of bone cancer and any metastasis. Surgeons will always try for limb-sparing - perhaps replacing the diseased bone with a titanium implant (prosthesis). But in some cases, amputation will be necessary. Further chemotherapy may be necessary after surgery.
Ewing’s sarcoma responds to radiotherapy and treatment may consist of a combination of radiotherapy, chemotherapy and surgery according to the extent and location of the disease.
Variations in the intensity of courses of treatment will be tailored to the Patient’s needs. This may include variations in the number of courses, and both the number of days in hospital and at home.
Usually there will be arrangements to have 24/7 contact availability with the hospital ward when the Patient is at home.
FIVE-YEAR SURVIVAL RATES.
NHS Choices offers the following advice:-
“The most important factor in determining the likely outlook for cases of bone cancer is whether the cancer has spread from the bone to other parts of the body (metastasis).
Health professionals use a general measurement of a "five-year survival rate" when describing cancer statistics. However, it's important to make clear that the five-year measurement is not an absolute measurement and it does not mean that people with bone cancer only have a five-year life expectancy.
Osteosarcoma : Six out of 10 people with localised osteosarcoma will live for at least five years after diagnosis and most of these people will be completely cured.
The outlook for metastatic osteosarcoma is much poorer as only 1 in 10 people will live for at least five years after being diagnosed.
Ewing's sarcoma : Seven out of 10 people with localised Ewing’s sarcoma will live for at least five years after diagnosis, and again, most of them will be completely cured.
Only 3 out of 10 people with metastatic Ewing’s sarcoma will live for at least five years after diagnosis.”
In general terms, the overall 5-year survival rate in the UK is considered to be 55%.
As found in research carried out by the University of Newcastle, this rate has not improved in the UK in 25 years – largely because there has been no investment in bone cancer research by the Government. During the same period, the most common childhood cancer – leukaemia – has seen its survival rate improve from 53% to 80% - thanks to Government funding into research.
For fuller information, it is recommended that you visit Patient.co.uk at – http://www.patient.co.uk/health/Cancer-of-the-Bone-(Primary).htm
or NHS Choices at
There is also a short video from NHS Choices at
The Guy Francis Bone Cancer Research Fund has, since 2002, raised over £180,000 for vital research into this devastating disease.
Raising awareness and campaigning by way of patient advocacy is also important. It is recognised that informing healthcare professionals about PBC will help reduce misdiagnosis and/or delayed diagnosis.
Patients and their families can also help by acting swiftly and being persistent if they think something is not right. Patients know their own body better than anyone else!
So please seek advice if you have a concern, after all –
“It probably isn’t .......... but it might be bone cancer”
THE IMPORTANCE OF RESEARCH.
The Guy Francis Bone Cancer Research Fund is passionate about raising funds in order to commission specific and relevant quality research to help to improve current practice and knowledge.
The Fund will also be following, with particular interest, the findings of current research and publications in areas such as:
- Mifamurtide (MEPACT). A breakthrough drug for some osteosarcoma patients?
- Fluoridation in water – is there a link with osteosarcoma in boys?
- Ewing’s sarcoma – why are the 5-year survival rates in Germany so much better?
- Titanium – what will the world shortage and soaring metal price mean for prosthetic manufacture?
- Social research – do we need a fresh look at the influence of lifestyle and environment on PBC patients?
- Connective tissue oncology