Ewing's sarcoma occurs in every bone of the foot. About one-third are located in the hindfoot, and 20% of the total occur in the calcaneous. The mean age at diagnosis of patients with tumors in the foot is 17 years. Patients normally have pain accompanied by significant diffuse swelling. The area may appear inflamed and a misdiagnosis of infection can be made. The white blood cell count, erythrocyte sedimentation rate and temperature may all be moderately elevated.
Patients have symptoms for an average of 14 months prior to diagnosis. Patients with hindfoot lesions have an average duration of symptoms of 22 months prior to diagnosis, whereas those with forefoot lesions have an average duration of symptoms of seven months.
Ewing sarcoma is the second most common tumor of the bone. It most often affects bones of the pelvis, the tibia, fibula, and femur, and can also begin in the soft tissues. This disease most often occurs in adolescents, with nearly half of cases arising between the ages of 10 and 20.
The radiologic appearance of Ewing's sarcoma in the foot is highly variable. The tumor may present as a small lytic lesion with a permeative appearance and no matrix or soft tissue calcification. In other cases the lesion causes permeative lysis accompanied by marked sclerosis. In an unusual case involving the distal fibula, the lesion appeared entirely cystic and was mistaken for a unicameral (simple) bone cyst.
Bone scan shows abnormal uptake, which may be intense. CT scan demonstrates the permeative destruction well. MRI is essential for surgical planning and demonstrates the soft tissue extent of the mass, which may be characteristically extensive.
Ewing's of the foot is a highly aggressive tumor with frequent metastasis. A complete cancer staging work-up is required and a carefully planned biopsy should be the last step in the sequence. Treatment consists of chemotherapy, radiation therapy, and surgical resection depending on stage and extent of disease.
Ewing's sarcoma in the foot with regional metastasis can be treated with radiation of the primary, resection and radiation for the metastasis plus chemotherapy in order to balance the positive and negative effects of treatment in the setting of a relatively poor prognosis.
Ewing's sarcoma can occur any time during childhood and young adulthood, but usually develops during puberty, when bones are growing rapidly. It is uncommon in African-American, African, and Chinese children.
The tumor may arise anywhere in the body, usually in the long bones of the arms and legs, the pelvis, or the chest. It may also develop in the skull or the flat bones of the trunk.
There are few symptoms. The most common is pain and occasionally swelling at the site of the tumor. Children may also break a bone at the site of the tumor after a seemingly minor injury (this is called a "pathologic fracture"). Fever may also be present.
The tumor often spreads (metastasis) to the lungs and other bones. Metastasis at the time of diagnosis is present in approximately one-third of children with Ewing's sarcoma. Rarely, Ewing's sarcoma can occur in adults.
What are the prognostic factors in Ewing sarcoma?
The extent of the disease is the most important factor in determining how the disease will progress.
Other factors that influence the prognosis include:
- Primary site (where the cancer began)
- The presence of metastasis (spreading to other areas)
- Size of the tumor
- Leukocyte count
- Sedimentation rate
- Serum LDH levels
What are the symptoms of Ewing's sarcoma ?
Symptoms of Ewing's sarcoma 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. Pain may become very intense when the tumour is located near important nerves, like in the sacrum, pelvis or spine. Swelling is often seen, especially when the log bones of the arms or legs are affected.
Sometimes the tumour can interfere with movement and can weaken the bones, occasionally leading to a fracture. Other symptoms of 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 Ewing’s sarcoma 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. Ewing's sarcoma is composed of small-blue-round cells, these can appear similar to other types of cancer so special stains and other laboratory tests are needed to make the diagnosis.
The doctor at the hospital will take your full medical history before doing a physical examination. This will include an examination of the painful bone to check for any swelling or tenderness. You will probably have a blood test done to check your general health.
A variety of tests and investigations may be needed to diagnose a Ewing’s sarcoma. An x-ray of the painful part of the bone is usually able to identify a tumour, although sometimes this can be difficult to see. Other tests are taken to check whether the cancer has spread elsewhere. These include a chest x-ray, bone scan, bone marrow aspirate, and an MRI or CT scan.
Ewing’s sarcoma is treated by a combination of treatments which may include chemotherapy , surgery and radiotherapy . Treatment will depend on individual factors such as the position and the size of the tumour.
As Ewing’s sarcomas are very rare they are usually treated at specialist hospitals by a team of doctors and other health professionals. This means that you may have to travel some distance to have your treatment.
Most people with Ewing’s sarcoma will receive chemotherapy to shrink the primary tumour and also treat any cancer cells that may have spread to other parts of the body. Between three and six months later, the tumour will be treated with either surgery to remove the tumour, or radiotherapy. Chemotherapy will often continue after either surgery or radiotherapy.
Major improvements in surgery for bone cancer have been made. In the past, it was often necessary to remove (amputate) the affected limb if Ewing’s sarcoma was found. Now, however, it is often possible just to remove the affected part of the bone and some of the healthy tissue around it. The bone is then replaced with a specially designed metal replacement (prosthesis) or a bone graft (bone taken from another part of the body). If the cancer affects a bone in or near a joint, the whole joint can often be replaced with an artificial one. These operations are known as limb-sparing surgery.
Unfortunately, it is not always possible to use limb-sparing surgery and occasionally an amputation may be the only way to treat the cancer. This is often the case when the cancer has spread from the bone into the nerves and blood vessels around it.
The type of surgery you have will depend on a number of factors. Your surgeon will discuss the different types of surgery with you in detail before any decision is made about your treatment. It is often helpful to talk to someone who has had the same operation that you are going to have. The medical and nursing staff will be able to arrange this for you. On some wards a special counsellor may be available to discuss any worries you may have.
Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. This is an important treatment for most people with Ewing’s sarcoma, as it can greatly improve the results of surgical treatment. It is often given before surgery and may shrink large tumours enough to avoid amputation. The course of chemotherapy will then continue after surgery to destroy any remaining cancer cells and stop the sarcoma from spreading outside the bone. This is known as adjuvant chemotherapy.
Chemotherapy can make you feel better by relieving the symptoms of the cancer, but it can sometimes have unpleasant side effects. Any side effects that do occur can often be controlled well with medicine.
Radiotherapy treats cancer by using high-energy rays to destroy the cancer cells, while doing as little harm as possible to normal cells. Radiotherapy is often very effective in treating Ewing’s sarcoma and this treatment is often used after chemotherapy and before or after surgery. In some cases, surgery is not possible, for example if the tumour is in the spine. In these cases, radiotherapy may be given in place of surgery.
Radiotherapy can cause general side effects, such as feeling sick (nausea) and tiredness .These side effects can be mild or more troublesome, depending on the strength of the radiotherapy dose and the length of your treatment. The radiotherapist will be able to advise you what to expect.
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