Ewing sarcoma is a rare cancer that develops in the bone or soft tissue. It is most commonly found in the bones of the legs, pelvis, ribs, spine and shoulders, and the soft tissue of the trunk, arms, legs, head and neck. However, it can develop anywhere in the body.
There are two other primary types of Ewing tumours that share genetic characteristics and abnormalities. The first is extraosseous Ewing tumour, which develops in the soft tissue surrounding the bone, such as cartilage. The second is peripheral neuroectodermal tumour (PPNET), which primarily affects soft tissues of the central nervous system (CNS). Almost all Ewing tumours involve a gene mutation on chromosome 22 in your DNA.
Ewing Sarcoma is more prevalent in males and is generally diagnosed children and teenagers. However, anyone can develop this disease.
Treatment
If a Ewing sarcoma is detected, it will be staged and graded based on size, metastasis and how the cancer cells look under the microscope. Staging and grading helps your doctors determine the best treatment for you.
Cancers can be staged using the TNM staging system:
- T (tumour) indicates the size and depth of the tumour.
- N (nodes) indicates whether the cancer has spread to nearby lymph nodes.
- M (metastasis) indicates whether the cancer has spread to other parts of the body.
This system can also be used in combination with a numerical value, from stage 0 – IV:
- Stage 0: this stage describes cancer cells in the place of origin (or ‘in situ’) that have not spread to nearby tissue.
- Stage I: cancer cells have begun to spread to nearby tissue. It is not deeply embedded into nearby tissue and has not spread to lymph nodes. This stage is also known as early-stage cancer.
- Stage II: cancer cells have grown deeper into nearby tissue. Lymph nodes may or may not be affected. This is also known as localised cancer.
- Stage III: the cancer has become larger and has grown deeper into nearby tissue. Lymph nodes are generally affected at this stage. This is also classified as localised cancer.
- Stage IV: the cancer has spread to other tissues and organs in the body. This is also known as advanced or metastatic cancer.
Cancers can also be graded based on the rate of growth and how likely they are to spread:
- Grade I: cancer cells present as slightly abnormal and are usually slow growing. This is also known as a low-grade tumour.
- Grade II: cancer cells present as abnormal and grow faster than grade I cancers. This is also known as an intermediate-grade tumour.
- Grade III: cancer cells present as very abnormal and grow quickly. This is also known as a high-grade tumour.
Once your tumour has been staged and graded, your doctor may recommend genetic testing, which analyses your tumour DNA and can help determine which treatment has the greatest chance of success. They will then discuss the most appropriate course of treatment for you.
Treatment is dependent on several factors, including location, stage of disease and overall health.
Treatment options for Ewing sarcoma may include:
- Chemotherapy.
- Surgery, potentially including:
- Tumour excision.
- Bone excision/osteotomy (removal of a small portion of affected bone).
- Limb amputation.
- Radiation therapy.
- Clinical trials.
- Palliative care.
For more information on treatment options, please refer to the Rare Cancers Australia treatment options page.
Risk factors
The risk factors for Ewing sarcoma are minimal. These risk factors include:
- Age – Ewing sarcoma is most common in children and teenagers.
- Ancestry – Ewing sarcoma is more prevalent in people with European ancestry.
- Having Fanconi anaemia.
Some of the information regarding risk factors was obtained from the Ewing Sarcoma Treatment (PDQ) page published by the National Cancer Institute.
Early symptoms
The symptoms of Ewing sarcoma will vary depending on location and whether is has metastasised (spread to other parts of the body). The most common symptoms of this disease are:
- A large lump, which may feel soft and warm.
- Pain and/or swelling in the bone and general area affected by the tumour.
- Fever.
- Easily broken bones.
- Fatigue.
- Unexplained weight loss.
Not everyone with the symptoms above will have cancer but see your general practitioner (GP) if you are concerned.
Some of the information regarding symptoms was obtained from the Ewing Sarcoma Treatment (PDQ) page published by the National Cancer Institute.
Diagnosis/diagnosing
If your doctor suspects you have a Ewing sarcoma, they may order the following tests to confirm the diagnosis and refer you to a specialist for treatment.
Physical examination
Your doctor will collect your overall medical history, as well as your current symptoms. Following this, they may examine your body to check for any abnormalities.
Imaging & blood tests
The doctor will take images of your body using magnetic resonance imaging (MRI), a computed tomography scan (CT scan), x-rays, bone scan and/or positron emission tomography (PET scan), depending on where it is suspected the cancer is. The doctor may also look at other parts of the body and looks for signs of metastasis. Additionally, a blood test may be taken to assess your overall health and help guide treatment decisions.
Bone marrow aspiration & biopsy
Once the location of the cancer has been identified, the doctor will perform a biopsy to remove a section of tissue using a needle. In most cases, this will be done by a bone marrow aspiration and biopsy. This process involves inserting the needle into the hipbone (or the breastbone in some cases) to remove samples of solid and liquid bone marrow. These samples will then be analysed for cancer cells.
Prognosis (Certain factors affect the prognosis and treatment options)
While it is not possible to predict the exact course of the disease, your doctor may be able to give you a general idea based on rate and depth of tumour growth, susceptibility to treatment, age, overall fitness and medical history. Generally, early-stage Ewing sarcomas have a better prognosis and survival rates. However, if the cancer is advanced and has spread, the prognosis may not be as good and there may be a higher risk of cancer recurrence. It is very important to discuss your individual circumstances with your doctor to better understand your prognosis.
References
Some references are to overseas websites. There may be references to drugs and clinical trials that are not available here in Australia.