A rare form of cancer, Ewing sarcoma affects the bones or soft tissues around the bones. It is most common among children and young adults, but can occur at any age. Pain, swelling, fever, and other symptoms can be associated with Ewing sarcoma. Ewing sarcoma is not inherited, but is caused by a genetic mutation that occurs after birth. It can spread to other parts of the body, such as the lungs, bone marrow, and lymph nodes.
The treatment for Ewing sarcoma depends on the location, size, and stage of the tumor, as well as the patient’s age and overall health. It can be treated using chemotherapy, surgery, or radiation therapy. A patient's prognosis for Ewing sarcoma depends on the response to treatment and the presence of metastases. This blog post discusses the causes, symptoms, diagnosis, treatment, and prevention of Ewing sarcoma.
Ewing sarcoma is caused by a genetic mutation that occurs in some cells, but its cause is not known. The mutation occurs when genetic material is exchanged between two chromosomes, usually chromosome 11 and chromosome 22. The new gene, EWSR1-FLI1, makes the cells grow and divide abnormally. It is not inherited from the parents, but occurs randomly after conception. A mutation can occur in any cell, but it is most likely to affect cells that form bones and soft tissues around them. Factors that increase the risk of developing Ewing sarcoma include:
Age: Ewing sarcoma is more common in children and young adults, especially between the ages of 10 and 20. However, it can occur at any age.
Sex: Ewing sarcoma is slightly more common in males than in females.
Ethnicity: Ewing sarcoma is more common in people of European descent than in people of African or Asian descent.
The symptoms of Ewing sarcoma depend on the location and size of the tumor, as well as the extent of its spread. The most common symptoms include:
Bone pain: This is the most common symptom of Ewing sarcoma. The pain may be constant or intermittent, and it may worsen at night or with activity. The pain may also cause limping or difficulty moving the affected limb.
Swelling and tenderness: The tumor may cause swelling and tenderness in the area of the bone or the soft tissue. The swelling may be visible or palpable, and it may feel warm and soft to the touch.
Fracture: The tumor may weaken the bone and cause it to break with minor trauma or without any apparent cause.
Fever: The tumor may cause a persistent low-grade fever that does not respond to antibiotics or other treatments.
Weight loss: The tumor may cause loss of appetite and weight loss without any intentional dieting or exercise.
Fatigue: The tumor may cause general weakness and tiredness that does not improve with rest or sleep.
Other symptoms: Depending on the location and spread of the tumor, Ewing sarcoma may cause other symptoms, such as:
Difficulty breathing, coughing, or chest pain if the tumor affects the lungs or the chest wall.
Numbness, tingling, or paralysis if the tumor compresses the nerves or the spinal cord.
Abdominal pain, nausea, vomiting, or constipation if the tumor affects the digestive organs or the abdomen.
Headache, dizziness, or vision problems if the tumor affects the brain or the skull.
The diagnosis of Ewing sarcoma involves a series of tests and procedures, such as:
In addition to looking for signs of Ewing sarcoma, such as pain, swelling, fracture, or fever, the doctor will also check for signs of metastasis, such as enlarged lymph nodes, liver, or spleen.
Imaging tests: The doctor will order imaging tests to visualize the tumor and its spread, such as:
Radiography: This test uses radiation to produce images of the bones and soft tissues. It can help determine a tumor's size and location as well as any bone damage or fracture.
A CT scan uses X-rays and a computer to create cross-sectional images of the body. It can provide more information about the tumor and its spread to other organs.
This test uses a strong magnetic field and radio waves to produce images of the body, which can reveal the extent and depth of a tumor, as well as its involvement of the nerves, blood vessels, and spinal cord.
A PET scan uses a radioactive substance that is injected into the bloodstream and accumulates in tumor cells. It can detect the metabolic activity of the tumor and its spread.
A biopsy is the only way to confirm the diagnosis of Ewing sarcoma. It involves taking a small sample of tissue from the tumor and examining it under a microscope.
Needle biopsy: The doctor inserts a thin needle through the skin and into the tumor to collect a small amount of tissue.
Surgical biopsy: The doctor makes a small incision in the skin and removes a larger piece of tissue from the tumor.
An analysis of the chromosomes of tumor cells can be done to detect the presence of the EWSR1-FLI1 gene, which is characteristic of Ewing sarcoma.
Blood tests: The doctor may order blood tests to check the levels of certain substances in the blood, such as:
A complete blood count (CBC) measures the number and type of red blood cells, white blood cells, and platelets. It may help detect anemia, infection, or bleeding issues.
Blood sedimentation rate (ESR): This test measures how quickly red blood cells settle at the bottom of a tube of blood. It can indicate inflammation or infection.
Test for lactate dehydrogenase (LDH): A test that measures the level of an enzyme released when cells are damaged or destroyed. It can be used to determine the extent of tissue damage or tumor growth.
Tests such as alkaline phosphatase (ALP) can indicate bone damage or liver problems by measuring the levels of an enzyme produced by the bones and liver.
The treatment of Ewing sarcoma depends on several factors, such as:
The location, size, and stage of the tumor.
The presence or absence of metastasis.
The patient’s age and overall health.
The patient’s preferences and goals.
The main treatment options for Ewing sarcoma are:
To shrink the tumor and prevent or treat metastasis, chemotherapy is usually administered before or after surgery or radiation therapy. Chemotherapy usually involves using drugs that kill cancer cells or stop them from growing. A variety of drugs can be used to treat Ewing sarcoma, including vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide. Chemotherapy can cause side effects, including:
Hair loss
Nausea and vomiting
Loss of appetite and weight loss
Mouth sores and infections
Low blood cell counts and increased risk of bleeding and infection
Kidney damage and hearing loss
Infertility and early menopause
The purpose of surgery is to remove the tumor and some surrounding normal tissue. Surgery is usually performed after chemotherapy has shrunk the tumor and made it easier to remove.
Local excision: This involves removing the tumor and a margin of normal tissue around it. This is the preferred method of treating Ewing sarcoma, since it preserves the function and appearance of the affected limb or organ.
As a result of amputation, the entire limb or part of it is removed. Usually, amputation is used when the tumor is too large or too close to vital structures, such as nerves or blood vessels, to be safely removed by wide local excision. Physical and emotional challenges can result from an amputation, including:
Pain and phantom sensations
Infection and wound healing problems
Difficulty with mobility and daily activities
Body image and self-esteem issues
Prosthesis: This is an artificial device that replaces a missing limb or part of it. Prosthesis can help restore the function and appearance of the affected limb. Prosthesis can be fitted and adjusted by a specialist called a prosthetist. Prosthesis can have advantages and disadvantages, such as:
Improved mobility and quality of life
Reduced pain and phantom sensations
Cost and maintenance
Need for regular replacement and adjustment
Possible complications, such as infection, skin irritation, or mechanical failure
To kill cancer cells or stop them from growing, radiation therapy uses high-energy rays or particles. Radiation therapy is usually given after surgery in order to eliminate any remaining cancer cells and prevent recurrences. As a palliative treatment to relieve pain and other symptoms, radiation therapy can also be given before surgery to shrink the tumor and make it easier to remove. Radiation therapy is administered in the following ways:
Radiation therapy with an external beam is most common for treating Ewing sarcoma. External beam radiation therapy involves delivering radiation from outside the body to the tumor and surrounding areas. It is usually given in daily sessions for several weeks. Side effects from external beam radiation therapy include:
Skin irritation and burns
Hair loss in the treated area
Fatigue and weakness
Nausea and vomiting
Diarrhea and bladder problems
Growth problems and bone deformities in children
Increased risk of secondary cancers
For Ewing sarcoma, internal radiation therapy is less common. By placing radioactive material inside or near the tumor, internal radiation therapy can deliver a higher dose of radiation to the tumor and spare normal tissues. Internal radiation therapy can be carried out by:
The procedure involves inserting radioactive seeds, wires, or rods into the tumor or cavity left by surgery. The radioactive material remains in place for a short period of time and then is removed.
Injection or ingestion of radioactive substances travels through the bloodstream and accumulates in tumor cells. After decaying, the radioactive substances emit radiation that kills the cancer cells.
It is a newer form of radiation therapy for Ewing sarcoma. It delivers radiation to the tumor using protons, positively charged particles. Due to its ability to stop at the tumor and avoid damaging the surrounding normal tissues, proton therapy is more precise and accurate than conventional radiation therapy. However, proton therapy does have some disadvantages, such as:
Reduced side effects and complications
Improved quality of life and survival
Limited availability and accessibility
Higher cost and insurance coverage issues
Since Ewing sarcoma is not caused by any environmental or lifestyle factors, there is no known way to prevent it. However, some general measures can help reduce the risk of cancer and improve overall health:
Avoiding tobacco and alcohol use
Eating a balanced and nutritious diet
Maintaining a healthy weight and physical activity level
Protecting the skin from sun exposure and avoiding indoor tanning
Getting regular check-ups and screenings
Following the doctor’s recommendations and treatment plans
Ewing sarcoma is a rare and aggressive type of cancer that affects the bones or soft tissues around the bones. It is most common in children and young adults, but it can occur at any age. The symptoms of Ewing sarcoma include pain, swelling, fever, and other symptoms. The disease can also spread to the lungs, bone marrow, and lymph nodes. It is not inherited, but a genetic mutation occurs after birth that causes Ewing sarcoma. There are many treatment options available for Ewing sarcoma, depending on the location, size, and stage of the tumor, as well as the age and overall health of the patient. There are three main treatment options for Ewing sarcoma: chemotherapy, surgery, and radiation therapy. It is not possible to prevent ewing sarcoma, but some general measures can help reduce the risk of cancer and improve overall health. The prognosis differs depending on the response to treatment and the presence of metastasis.
