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Bone CancerIntroduction / About this FAQ
The contents of this document have been prepared by an international panel of experts to answer some of the frequently asked questions (FAQ) about bone cancer. The project has been
supported by The Scientific Committee of SIOP (International Society of Paediatric Oncology).
Please note:
- The main focus of the FAQ is on cancer that has started in the bone (primary bone cancer) rather than cancer that started in another part of the body and later spread to the bone.
- The FAQ is intended to provide a supplementary source of information about bone cancers. If you are a patient or parent of a child with bone cancer your doctor should be able to provide you with the information which is most relevant to you.
- It is not designed to provide medical advice and cannot be a substitute for professional medical services.
- The FAQ has a section on general questions about bone cancer and separate pages about Ewing’s sarcoma and Osteosarcoma.
| Dr G Bacci | Prof. S Burdach | SJ Cotterill | Prof. AW Craft |
| Mr R Grimer | Prof H Jurgens | Prof R Kotz | Dr H Kovar |
| Dr PA Meyers | Dr O Oberlin | Dr P Picci | Dr G Saeter |
| Dr D Spooner |
What is osteosarcoma ?
Osteosarcoma is a disease in which cancer cells are found in the bone. It is the most frequent type of bone tumour and is most common between the ages of 10 to 25. In most cases it is found in the bones around the knee. It is usually located in the growing ends of the bone (metaphysis). It is sometimes also called "osteogenic sarcoma" which literally means a bone forming cancer.The most common early signs of osteosarcoma are pain and swelling. Like other sarcomas, osteosarcoma can spread to other parts of the body. Even when the tumour is detected at a very small size, there may be microscopic spread. For this reason, osteosarcoma always requires treatment to the whole body. This treatment includes chemotherapy. Chemotherapy is intended to destroy the tumour cells which have spread to the rest of the body and to shrink the main mass of tumour cells. Successful treatment also requires another form of treatment to the main mass of tumour. This is usually surgery to remove the main tumour.
What are the symptoms of osteosarcoma?
Symptoms of osteosarcoma vary from person to person and depending on the location and size of the cancer. The most common symptoms are pain and swelling or tenderness in the affected area. As this disease affects active young people symptoms are often attributed to over-stress or trauma. However, with osteosarcoma pain tends to increase over time. Any long lasting pain should be reported to a doctor.
Sometimes the tumour can interfere with movement and can weaken the bones, occasionally leading to a fracture. Other symptoms of the cancer may include tiredness, fever, weight loss, and anaemia. None of these symptoms is a sure sign of cancer; if you suspect you have a health problem consult your doctor.
How is osteosarcoma diagnosed ?
If a bone tumour is suspected the doctor will do a complete medical examination. This may include a blood test as bone tumours can be associated with increased levels of certain enzymes in the blood. The doctor may also recommend X-rays and other scans of the bone(s), if X-rays and scans suggest that a tumour might be present then a biopsy (removal of a sample of tissue) will be performed. A pathologist will then examine the cells to determine whether it is cancerous, and if so what type of cancer it is. Under the microscope malignant bone formation is a sign of osteosarcoma. Osteosarcoma can appear similar to other types of cancer so special stains and other tests may be needed to make the diagnosis.What is the treatment for osteosarcoma ?
Modern treatments are based on chemotherapy combined with local therapy (usually surgery or sometimes radiotherapy to the main tumour):
- chemotherapy (using drugs to kill cancer cells)
- surgery (to take out the tumour in an operation)
- radiotherapy (using high-dose x-rays to kill cancer cells)
Most osteosarcomas are treated with surgery to remove the tumour and a margin of the healthy tissue around the cancer. The type of surgery will depend on the site and location of the main tumour and other individual factors. Sometimes all or part of an arm or leg may have to be removed (amputated) to make sure that all of the cancer is removed. In other cases limb-sparing surgery may be possible, where the tumour is removed without an amputation. In limb-sparing operations the bone is taken out and replaced by an artificial device (endoprosthesis) or bones from other places in the body (bone graft).
Chemotherapy is also given to kill malignant cells that may be circulating around the body. The chemotherapy is usually a combination of different drugs. Treatment of bone cancers is complex and involves a team of different specialists usually within an institution that is experienced in treating these types of cancers.
What is the chance of recovery ?
Overall, the chance of recovery (prognosis) for osteosarcoma has dramatically improved since the development of modern chemotherapy.The chance if recovery will depend on a variety of influences; if the cancer has spread, the size of the tumour, location, type of osteosarcoma, the person's general health and other individual factors. Also important is how much of the main tumour can be taken out by surgery and/or how the tumour responds to chemotherapy.
What causes osteosarcoma ?
The vast majority of osteosarcomas are so-called "random cases", where no specific causes or predisposing risk factors can be identified.In young people, the development of the tumour appears to be in some way related to periods in life with rapid bone growth, hence the average for tumour development is 14-16 years. The relationship to bone growth may also be part of the explanation why osteosarcoma is slightly more common in boys than in girls. However, osteosarcoma remains an extremely rare tumour in all groups of the population, and there is no extra cause for concern in rapidly growing teenagers. The relationship between bone growth and osteosarcoma formation is thought to be due to an increased vulnerability of rapidly growing cells to damage caused by chance or by as yet unidentified factors.
Sometimes the person with osteosarcoma or the parents relate a previous injury or trauma to the development of osteosarcoma. However, medical research has not found any relationship between such injury and the risk of subsequent osteosarcoma development.
In very rare cases osteosarcoma may be related to rare genetic abnormalities or to pre-existing bone disease. The most acknowledged genetic risk factor is hereditary retinoblastoma, a tumour of the eye which develops in early childhood. In these instances the inherited gene is of a nature that predisposes to both the eye tumour and to osteosarcoma.
What sort of function can I expect with an amputation ? (leg)
The degree of function that most people can expect with an amputation depends very much on the individual and the level of the amputation.The type of amputation will depend on the location of the tumour and other factors.In general, all amputations of the upper leg result in significant loss of function. Lower limb amputations tend to produce better function the further down the limb they are. Most patients with below knee amputations have very good functional outcomes with the ability to play sport, run and swim with few problems. Above knee amputations are more variable and will depend to a certain extent upon the remaining length of the femur. The ideal amputation is 15cm above knee and amputees at this level will usually be provided with an artificial limb which will allow them to walk normally and, in some instances play sports. Amputations through the upper leg, through the hip joint or hindquarter amputations are less satisfactory and some patients will find that they prefer not to wear an artificial limb as they are more mobile with crutches.
What are the long-term problems of endoprosthesis ?
An endoprosthesis is an artificial bone replacement within the body, for example a metal rod replacing a bone, while leaving the surrounding tissues (muscle, skin, blood vessels, nerves etc) in place. Endoprostheses are often used in limb-sparing surgery for bone tumours. Some people have no problems with their endoprosthesis. However, there are potential long-term problems, these will vary from person to person.Like any other prosthesis the tumour endoprosthesis can have problems with wear. In the case of a knee amputation there is usually a hinge necessary to compensate for muscle and ligament resection. The axis is under stress and may have to be revised after wear. Therefore, after 5 to 10 years a rebrushing of polyethylene parts can be necessary. In case of metal-metal axis there can be some metallosis, but duration of the material is much longer and may last 20 years or more.
Another problem can be late infections. This can require further surgery to revise the prosthesis, and in the majority of cases a one-step revision is possible.
Also, the long-term durability of stems and body of the prosthesis can be problematic. Cemented as well as non-cemented stems may break after some time depending on the activity, weight and height of the patient. Also, stress shielding problems can occur at the anchorage which in turn may cause fracture of the stem. Sometimes motion-associated soft tissue corrosion may occur. In children, a thick fibrous tissue may develop around the prosthesis possibly inhibiting motion after some years which requires revision and resection of the fibrous tissue sleeve.
Will I be sterile after chemotherapy ?
boys and men.Most drugs will have no effect at all on your fertility, however some may reduce the number of spermatozoa. This greatly depends on the class of chemotherapy drugs used. The main drugs applied nowadays for osteosarcomas are methotrexate, cisplatinum, adriamycin, ifosfamide. The « BCD » combination has also been used and consisted of bleomycin, cyclophosphamide and actinomycin D. Methotexate even given at high dose, actinomycin D, adriamycin and bleomycin have no effect on gonads. Cyclophosphamide belongs to the alkylating family of drugs and the induction of gonadal dysfunction induced by this drug has been reported for many years. However, it became known that this toxicity is related to the cumulative dose. It is rare for doses lower than 3 - 4 g/m2 but progressively increases with a higher risk for dose greater than 9 g/m2. Less is known about the late effects of ifosfamide or cisplatinum but it seems to have less consequences. If the chemotherapy does cause infertility, some patients will remain infertile after their treatment has stopped, but some find their sperm returns to normal levels, sometimes after many years. If the chemotherapy is started after puberty it is possible to bank the sperm, a procedure that freezes sperm for future use.
girls and women
During chemotherapy, the monthly periods may become irregular and eventually stop completely and most often will return to normal after the treatment finishes.
But most drugs will have no effect at all on your later fertility. In women being given chemotherapy during adulthood, the younger they are, the more likely they are to keep being able to have
children. The follow-up of women treated during childhood or adolescent support the fact that the risk of permanent ovarian damage is very low. However, there may be a possibility that menopause
occurs earlier than usually for them.
men and women.
Reports of pregnancy after cytotoxic chemotherapy have suggested that there is no increased incidence of fetal wastage and malformations or of cancer over that of the general population.
Where can I get more information ?
Please note: the authors of this FAQ cannot be held responsible for information from other sources.
- Your Doctor
- If you are a patient or parent of a child with bone cancer your medical team should be able to
provide you with the information which is most relevant to you. Don't be afraid to ask ! Some people
find it useful to write down questions before seeing their doctor.
- Telephone Helplines
USA - Cancer Information Service
List of Helplines Around the World- Other Sources
- There are various sources of information about bone cancer and cancer organisations on the Internet. However, the quality of information can vary. Lists of links by topics can be found in:
Children's Cancer Web
Guide to Internet Resources for Cancer
Osteosarcoma
Your Feedback
With regret it is not possible to answer individual medical queries; see Where can I get more information?. Use the form on our WordPress site for your feedback and suggestions for the FAQ.
If you have or suspect you may have a health problem you should consult your doctor as soon as possible .
The FAQs were last updated in 2004, but remain relevant.
