Agency Links:    Home   Contact Us    Compliments & Complaints   Help    Site Map
Link to Homepage

Patient/Public Info  |  Regional Services  |  Health Professionals Info  |  About BCCA  |  Research  |  Donating

Sarcomas

Reviewed July 2014

This information should not be used for self-diagnosis or in place of a qualified physician’s care.


      

Table of Contents

Bone sarcomas   Soft tissue sarcomas   All sarcomas
What causes it and who gets it?  
Can I help to prevent it?
Screening for sarcomas
Signs and Symptoms
Diagnosis
Types and Stages
Treatment 
What causes it and who gets it? 
Can I help to prevent it?
Screening for sarcomas
Signs and Symptoms
Diagnosis
Types and Stages
Treatment
Follow-up after treatment
Living with cancer
Library Resources
Recommended Websites
Can I help with research at BCCA?

Bone Sarcomas

  • Bone sarcomas are also known as primary bone cancer. They are cancers that start in the bone.
  • For cancer that has spread to the bone from another part of the body, please refer to information on secondary bone cancer and the type of cancer that was first diagnosed.
  • The 206 bones in the human body provide structure and protection.
  • Bone sarcomas can occur in any bone in the body but are commonly found in the bones in arms and legs.

What causes it and who gets it? 
Listed below are some of the known risk factors for this cancer.  Not all of the risk factors below may cause this cancer, but they may be contributing factors.

  • Patients who have received past treatment with radiation therapy have a higher risk of developing bone sarcomas.
  • Patients who have Paget's disease are at an increased risk for to developing osteosarcoma. Paget’s disease is a non-cancerous bone condition in which parts of the skeleton become overactive, break down and then re-grow at an abnormally fast rate.
  • Bone sarcomas can sometimes occur because of genetic abnormalities in tumour suppressor genes, such as the retinoblastoma (Rb) gene or the p-52 (p-glycoprotein) gene. Tumour suppressor genes keep cells from dividing too quickly. Abnormalities in these genes could cause cells to grow out of control and may lead to cancer.
  • Bone sarcomas may develop in patients who have fibrous dysplasia (a bone disease that destroys and replaces normal bone with fibrous bone tissue).
  • Patients with familial endochondromatosis (a benign growth found in the cartilage of hands and feet) may also develop bone sarcomas.
  • Statistics
    • Primary bone cancers (cancer that starts in the bone) are very rare - less 0.5% of all cancers. It is much more common for other types of cancer to spread to the bone.
    • Most recent available statistics:
      • B.C. statistics (2011)
        • 42 new cases diagnosed – 17 female, 25 male
        • 17 deaths – 6 female, 11 male
      • Canada 

Can I help to prevent it?

  • There are no known prevention measures for bone sarcomas.

Screening for this cancer

  • No effective screening program exists for this cancer yet.

Signs and Symptoms

  • Diagnosing bone sarcomas is difficult because the symptoms are similar to those of bone injuries, bursitis, arthritis or non-cancerous bone tumours.
  • The symptoms vary according to the type and location of the sarcoma. The following list contains some of the general symptoms of bone sarcomas:
    • Pain in a bone or joint
    • Swelling
    • Fever
    • Tumours can weaken bones and cause fractures.

Diagnosis
These are tests that may be used to diagnose this type of cancer.  

  • Physical examination.
  • An x-ray to help locate the tumour.
  • A chest x-ray is used to determine if the cancer has spread to the lungs.
  • A computerized tomography scan (CT Scan) produces a three dimensional image of the tumour and also screens for spread to the lungs if the chest X-ray was normal.
  • A magnetic resonance imaging scan (MRI) produces detailed images of the tumour.
  • A biopsy requires the removal of a small amount of tissue for examination under a microscope to determine if the tumour is cancerous. This step in the diagnosis should be approached with caution, and should be done in consultation with an orthopedic oncologist.
  • A routine bone scan is done for patients with osteosarcoma or Ewing’s sarcoma at diagnosis, at completion of their therapy and at recurrence.

For more information on tests used to diagnose cancer, see our Recommended Websites, Diagnostic Tests section.

Types and Stages

Types of Bone Sarcomas

  • These tumours are named for the tissue type which they most resemble. For example, tumours that resemble bone-producing cells are called osteosarcoma; those looking like fat cells are called liposarcoma.

Osteosarcoma

  • Osteosarcoma is the most common bone sarcoma.
  • It occurs most often in the growing ends of the long bones of the arms and legs (such as at the knees and shoulders).
  • Osteosarcoma is most commonly found in adolescents. It is slightly more common in males than in females and may be related to the adolescent growth spurt.
  • Osteosarcoma also affects older adults.
  • Osteosarcoma can spread to the lungs and to other parts of the body through the bloodstream.
  • Lymph node involvement is unusual.
  • Surgery to remove the tumour completely is the primary method of treatment. If possible, the part of the bone that has been removed will be reconstructed to avoid amputation.
  • Chemotherapy is used to stop the cancer from spreading to the lung and other organs in the body.
  • Radiation therapy is rarely used.

Chondrosarcoma

  • Chondrosarcoma is a type of bone tumour that grows in the cells that produce cartilage.
  • It occurs most often in older adults.
  • Bone swelling and pain are the most common symptoms.

Ewing's Sarcoma

  • Ewing’s sarcoma is a family of tumours that occur in the bone or soft tissue.
  • Ewing tumour of the bone is a specific type of Ewing’s sarcoma.
  • Ewing’s sarcoma is a very aggressive type of bone sarcoma.
  • Chemotherapy is used to stop the cancer from spreading to other organs in the body.

The following bone sarcomas are less common:

Chordoma

  • This is a rare tumour arising at the end of the spinal cord or near the base of the skull.
  • Pain near the end of the spinal cord is most typical for tumours in that area.
  • Tumours growing at the base of the skull produce symptoms that affect the cranial nerves, most commonly the nerves of the eyes.
  • Symptoms may last for many months before the diagnosis is clear.
  • Tumours grow very slowly and spread by local invasion.
  • Chordomas are generally treated by surgery and radiation.

Malignant Fibrous Histiocytoma (MFH) of the Bone

  • Malignant Fibrous Histiocytoma (MFH) is a rare malignant tumour of the bone.
  • In some situations, MFH arises out of osteosarcoma.
  • Osteosarcoma and Malignant Fibrous Histiocytoma are generally treated in the same way.

Peripheral Neuroectodermal Tumour (PNET)

  • Peripheral Neuroectodermal Tumours (PNET) are more common in childhood.
  • The tumours have the same genetic change as Ewing's sarcoma of the bone.

Myeloma, Lymphoma

  • Myelomas and lymphomas are cancers of the bone marrow, which are discussed in their own sections. See the Types of Cancer pages on Myelomas and Lymphomas.

Stages
Staging describes the extent of a cancer. The TNM classification system is used as the standard around the world.  In general a lower number in each category means a better prognosis. The stage of the cancer is used to plan the treatment.

G describes how the tumour cells look under the microscope
T describes the site and size of the main tumour (primary)
N describes involvement of lymph nodes
M relates to whether the cancer has spread (presence or absence of distant metastases)

        Stage I The tumour is low grade (the tumour looks more like normal bone). The tumour is any size. It has not spread to lymph nodes or distant sites. The cancer does not look different from normal tissue.
Stage II The tumour is high grade (the tumour looks very different from normal tissue.).  The tumour is any size. The cancer has not spread to lymph nodes or distant sites.
Stage III     The tumour is high grade and has spread to one or more sites within the bone.
Stage IV   The cancer has spread to lymph nodes or to distant sites.
  • G1  Low grade
  • G2  Intermediate grade – treated as high grade
  • G3  High grade
  • T1  Tumour is 8cm or less in greatest dimension
  • T2  Tumour more than 8cm in greatest dimension
  • T3  Discontinuous tumours in the primary bone site
  • NX  Regional lymph nodes cannot be assessed
  • N0  No regional lymph node metastasis
  • N1  Regional lymph node metastasis
  • M0  No distant metastasis
  • M1  Distant metastasis
  • M1a Lung
  • M1b Other distant sites

Treatment                                                                                               Top
Cancer therapies can be highly individualized – your treatment may differ from what is described below.

  • The complete surgical removal of the tumour is the most important treatment.
  • Many fast-growing bone tumours are also treated with chemotherapy which is often given before surgery.

Go to Followup after Treatment.

~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~

Soft Tissue Sarcomas                                                                              Top
  • Soft tissue connects, supports and surrounds the organs and structures within the body.
  • Soft tissue consists of muscles, joints, fat, blood vessels, tendons, synovial tissues (tissues around joints) and nerves.
  • Soft tissue sarcomas form in the soft tissues anywhere in the body but are most commonly found in the arms and legs.

What causes it and who gets it?
Listed below are some of the known risk factors for this cancer.  Not all of the risk factors below may cause this cancer, but they may be contributing factors.

  • In most cases, there is no identifiable cause of the soft tissue sarcoma.
  • Sarcomas can sometimes occur because of genetic abnormalities in tumour suppressor genes, such as the p-52 (p-glycoprotein) gene or retinoblastoma gene. Tumour suppressor genes keep cells from dividing too quickly. Abnormalities in these genes could cause cells to grow out of control and may lead to cancer.
  • Previous chemotherapy can increase a patient’s risk of developing soft tissue sarcoma.
  • Patients who have received past treatment with radiation therapy have a higher risk of developing sarcoma in that area.
  • It is suspected that large amounts of occupational exposure to herbicides such as phenoxyacetic acids, wood preservatives such as chlorophenols, or plastic manufacturing chemicals such as vinyl chloride may increase the risk of developing soft tissue sarcomas.
  • Exposure to vinyl chloride, a chemical used in plastic manufacturing, can increase the risk of developing soft tissue sarcoma.
  • Statistics - most recent available statistics:
    • BC - There were 133 new cases of soft tissue cancers diagnosed in 2007.
    • Canada - There were 1050 new cases of soft tissue cancers diagnosed in Canada in 2006.

Can I help to prevent it?

  • Limit occupational exposure to chemicals (such as phenoxyacetic acids, chlorophenols and vinyl chloride). 

Screening for this cancer

  • No effective screening program exists for the cancer yet.

Signs and Symptoms

  • Symptoms are dependent on the location of the tumour.
  • The first symptom that is usually noticed is a painless lump growing in the muscle or under the skin.
  • If the tumour is pressing against nearby organs, nerves or muscles there may be more symptoms such as:
    • Weight loss
    • Fever

Diagnosis
These are tests that may used to diagnose this type of cancer.

  • Physical examination.
  • A chest x-ray is used to determine if the cancer has spread to the lungs.
  • A computerized tomography scan (CT Scan) produces a three dimensional image of the tumour and screens for spread to the lungs if the chest X-ray was normal.
  • A magnetic resonance imaging scan (MRI) produces detailed images of the tumour.
  • A biopsy requires the removal of a small amount of tissue for examination under a microscope to determine if the tumour is cancerous. This step in the diagnosis should be approached with caution, and should be done in consultation with a surgical oncologist.
  • A routine bone scan is done for patients with rhabdomyosarcoma or soft tissue variants of ewing's/ peripheral neuroectodermal tumour (PNET). 
For more information on tests used to diagnose cancer, see our Recommended Websites, Diagnostic Tests section.

Types and Stages

Types of Soft Tissue Sarcomas

  • The tumours are divided into groups named for the tissue type that the sarcoma cells most closely mimic.

Fibrosarcomas

  • Fibrosarcomas often occur around scars, muscles, nerves, tendons and around the lining of the bone.
  • The cancer can spread through the bloodstream to the lungs.
  • It is most frequent in older adults.

Liposarcomas

  • These tumours develop in the fat tissue of the thigh, groin, buttocks area, or abdomen.
  • These tumours may be large but their large size does not necessarily mean that they are fast-growing.
  • They are different from and do not arise from lipomas (non-cancerous fatty tumours).
  • Liposarcomas are most common in older adults.
  • A very aggressive round cell variant may be treated with adjuvant chemotherapy along with surgery and radiation.

Rhabdomyosarcomas

  • Rhabdomyosarcoma is the most common soft tissue sarcoma of childhood. It generally affects children between the ages of two and six.
  • Rhabdomyosarcomas can spread quickly through the bloodstream to distant parts of the body.
  • Rhabdomyosarcomas are divided into subtypes:
    • Embryonal – occurs in the head, neck and eye
    • Embryonal botryoid – occurs in the genito-urinary tract
    • Alveolar – occurs in the muscles of arms and legs
    • Adult pleomorphic or anaplastic - occurs in older kids and adults patients, usually in the muscles of arms and legs.
  • Treatment includes surgery, radiation and chemotherapy.

Leiomyosarcomas

  • Leiomyosarcomas are tumours of the smooth muscle that commonly arise in the uterus or in blood vessels.

Angiosarcomas and Lymphangiosarcomas

  • Angiosarcomas arise in vascular tissue and Lymphangiosarcomas arise in the lymphatic system.
  • These cancer cells easily spread into the bloodstream.
  • Lymphangiosarcoma may be seen in patients with chronic lymphedema.

Synovial Cell Sarcomas

  • Synovial Cell Sarcoma usually occurs in tissue next to the joints, especially in the knee, ankle and hand.
  • These tumours, as with other soft tissue sarcomas, can occur anywhere in the body, even in the heart.
  • Occasionally they may spread to the skin, notably the scalp.
  • Spread to the lymph nodes is uncommon.

Malignant Peripheral Nerve Sheath tumour (MPNST) (previously called Neurofibrosarcoma)

  • MPNST develops in the cells that surround a nerve.
  • It may arise in patients with neurofibromatosis (a genetic disorder of the nervous system that effects the development of nerve cell tissues).
  • As the tumour grows it may cause pain or dysfunction.

Malignant Fibrous Histiocytoma (MFH)

  • Malignant Fibrous Histiocytoma is a term that describes a high grade soft tissue tumour that does not look like any specific subtype of sarcoma.
  • It can be found anywhere in the body.
  • In superficial sites such as the skin, it may behave like a non-cancerous tumour despite fast growing cells, but must be adequately removed with a cancer-type surgery.
  • When MFH occurs deep within body cavities or muscles it may be very fast growing.

Gastrointestinal Stromal Tumour (GIST)

  • GIST is one of the most common types of sarcoma with approximately 50 cases per year in BC.
  • Patients may have symptoms such as a lump in their abdomen or very low red blood cells counts (anemia). 
  • Sometimes GIST’s may be discovered when a patient is having a scan or operation for a different reason.
  • Gastrointestinal Stromal Tumours were previously called leiomyosarcoma of the gastrointestinal tract but they are now believed to be related to the cells which control normal bowel contractions.
  • GIST may arise anywhere within the abdomen, but most commonly from the stomach and small bowel.
  • The cells behave like normal cells and grow at a slow rate. However, it may be very hard to prevent recurrence even if the tumour has been surgically removed.
  • Spreads to liver first rather than to the lungs.
  • Drug treatment may be used for patients with high risk tumours and for patients who have had spread of the cancer to other sites.

Kaposi's Sarcoma                                                             

Stages
Staging describes the extent of a cancer. The TNM classification system is used as the standard around the world.  In general a lower number in each category means a better prognosis. The stage of the cancer is used to plan the treatment.

G describes how the tumour cells look under the microscope
T describes the site and size of the main tumour (primary)
N describes involvement of lymph nodes
M relates to whether the cancer has spread (presence or absence of distant metastases)

Treatment                                                                                  Top        
Cancer therapies can be highly individualized – your treatment may differ from what is described below.

  • The complete surgical removal of the tumour is the most important treatment.
  • For tumours of arms, legs or superficial trunk, radiation therapy may be used before or after surgery to decrease the chance of tumour recurrence, particularly for quick-growing tumours.
  • Surgery to the lung may be offered to patients where the cancer has spread to the lung.

~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~

The following information is for all sarcomas.

Follow-up after Treatment
 

  • Guidelines for follow-up after treatment are covered on our website. 
  • You will be returned to the care of your family doctor or your specialist for regular follow-up. If you do not have a family physician, please discuss this with your BC Cancer Agency oncologist or nurse.
  • Follow-up testing is based on your type of cancer and your individual circumstances.
  • The BCCA Survivorship Research Centre focusses on the issues that cancer survivors can face.

Living with Cancer

We know that cancer and its treatment can present unique challenges: from everyday concerns like diet, money and housing, to emotional concerns. The needs of our patients and their families and friends can be very diverse. Please explore this very useful part of our website. Here is some of what's included in this section:

Library Resources

  • The BC Cancer Agency Library has many resources about cancer, coping, talking to children and more. Please visit the Library in your Centre, call a librarian, or visit the Library online to see the many resources available.
  • Pathfinders are a good starting point to resources on types of cancer and living with cancer. They list books, videos, pamphlets, websites and support services.

Recommended websites 
The following websites have been selected and evaluated by the BC Cancer Agency.

Bone
Sarcomas
Websites for cancer survivors, and how to stay healthy after treatment.

Videos
View BC Cancer Agency videos on cancer-related topics.

Can I help with research at BCCA?
Patients play a vital role in research to fight cancer. Here are a few ways that you can help:



This information has been reviewed and approved by a member of the Musculoskeletal and Sarcoma Tumour Group.