Ewing sarcoma symptoms

Symptoms of Ewing sarcoma depend on where the tumor is located. Symptoms might include:

• Swelling or a lump around the tumor site
• Fever
• Bone pain
• Limping or problems walking
• Bone that breaks without a reason

Sarcomas are cancer found in the bone and soft tissue of the body. Watch this video to learn about sarcoma symptoms, diagnosis, and treatment in children.

Pain can get worse over weeks or months. It may even cause your child to wake up at night.

The signs and symptoms of Ewing sarcoma often start out like normal childhood aches and pains. This means that it can take a while before Ewing sarcoma is diagnosed.

Ewing sarcoma risk factors

We do not know why children and young adults develop Ewing sarcoma. But certain factors can increase the risk. These factors include:

• Age: About half of Ewing sarcoma tumors occur in children and teens 10–20 years of age.
• Sex: Ewing sarcoma occurs slightly more often in males than females.
• Race: Whites have a higher risk of developing Ewing sarcoma than Blacks and Asians do.
• Prior exposure: Cancer survivors who had radiation therapy have a higher risk of developing Ewing sarcoma.

Ewing sarcoma does not seem to be passed down in families.

Ewing sarcoma diagnosis

Doctors use several procedures and tests to diagnose Ewing sarcoma. These include:

• A health history, physical exam, and blood tests to learn about symptoms, general health, past illnesses, and risk factors. 
• A Complete Blood Count 
• A test to check the level of lactate dehydrogenase (LDH). This substance in the blood may be high in patients with Ewing sarcoma.
• Imaging tests such as: 
• X-Ray 
• A CT scan of the chest to see if Ewing sarcoma has spread to the lungs
• An MRI of the primary (first) tumor to evaluate the tumor and help plan for surgery
• Full-body imaging using bone scans  or PET-CT to look for cancer in other bones and other areas of the body to see if cancer has spread to the inside part of the bones (bone marrow)
• A biopsy of the mass to check for cancer

Ewing sarcoma staging

Although other cancers are classified as stages 1–4, Ewing sarcoma is classified as either localized or metastatic.

Localized means there is a tumor in only 1 place in the body.

Metastatic means the cancer has spread to 1 or more places. These might include:

• Lungs
• Lymph nodes
• Bones
• Bone marrow

About 25% of patients have metastatic disease when they are diagnosed. In about half of those patients, the disease has spread to the lungs.

Ewing sarcoma treatment

Ewing sarcoma is usually treated with chemotherapy followed by surgery and more chemotherapy. Radiation therapy may be used in place of surgery or added to the treatment.

Your child may be treated with 3–4 months of chemotherapy before having surgery, radiation or both. Your child may need more chemotherapy after surgery or radiation therapy. The typical length of treatment for Ewing sarcoma is about 8–9 months 

Ewing sarcoma prognosis

Survival of Ewing sarcoma depends on several factors:

• Size and location of the tumor
• Large tumor size is linked to worse survival.
• Cancer in the pelvis, ribs, or spine is harder to treat.
• Patients with Ewing sarcoma tumors in soft tissue without bone involvement tend to have improved survival compared to those with a tumor in the bone.
• Patient age and gender
• Patients under age 15 tend to have improved survival than older patients.
• Girls tend to have a better prognosis than boys.
• Cancer that has spread to other parts of the body (metastatic)
• Patients with metastatic tumors in the lungs do better than patients with metastatic tumors in the bones or lymph nodes.
• How the tumor responds to chemotherapy or radiation therapy
• Whether the cancer has come back

Patients who have chemotherapy and a single, localized Ewing sarcoma tumor that can be completely removed with surgery have a 70% chance of long-term cure in the United States.

Patients who have cancer that has spread to distant parts of the body at diagnosis have a 15–30% chance of survival, depending on where the cancer has spread.

The prognosis for patients with recurrent Ewing sarcoma is poor. The chance of cure after the cancer has returned (relapsed) is around 10–15% in the U.S.

Patients with recurrent Ewing sarcoma often develop new tumors within the first 2 years of diagnosis. This most often occurs as new growths in the lungs.

Patients who have early recurrence (their Ewing sarcoma comes back within 2 years of diagnosis) are much harder to cure than those who have later recurrence.

Balancing quality of life with cancer-directed therapy is important. Talk to your care team about problems to expect and how to help manage them. Palliative care can help families manage symptoms, promote quality of life, and make informed decisions.

Life after Ewing sarcoma 

Monitoring for Ewing sarcoma recurrence

Ewing sarcoma can come back years into survivorship. But it is most common within the first 2 years after treatment ends. The tumor can come back in the same place or in another part of the body.

Your child will need follow-up care to screen for recurrence for a long time after treatment ends. The care team will suggest types of tests and how often they should be done. Imaging tests usually include:

• CT scans of the lungs
• X-rays or MRI of the primary tumor site
• PET or bone scans

If your child has had Ewing sarcoma in the bone marrow, the care team may also suggest bone marrow tests.

Diagnostics of Ewing Sarcoma

The program includes:

• Initial presentation in the clinic
• clinical history taking
• review of medical records
• physical examination
• laboratory tests:

• complete blood count
• biochemical analysis of blood
• inflammation markers (CRP, ESR)
• indicators of blood coagulation
• tumor markers

• CT/MRI local
• high-resolution computed tomography (HR-CT)  of chest
• Renal scan
• Bone Scan
• Bilateral BMA&B
• Echocardiography
• biopsy with histological and immunohistochemical study 
• nursing services
• consultation of related specialists
• treatment by chief physician and all leading experts
• explanation of individual treatment plan (the cost of medicines is not included)

Required documents

• Medical records
• MRI/CT scan (not older than 3 months)
• Biopsy results (if available)

Type of program : Outpatient

Expected duration of the program: 15 days

Chemotherapy of Ewing sarcoma

The program includes:

• Initial presentation in the clinic
• clinical history taking
• review of medical records
• physical examination
• laboratory tests:

• complete blood count
• general urine analysis
• biochemical analysis of blood
• TSH-basal, fT3, fT4
• tumor markers
• inflammation indicators
• indicators of blood coagulation

• courses of chemotherapy
• nursing services
• consultations of related specialists
• treatment by head doctor and leading experts
• explanation of individual treatment plan

How program is carried out

During the first visit, the doctor will conduct a clinical examination and go through the results of previous laboratory tests and instrumental examinations. After that, you will undergo an additional examination, including laboratory assessment of liver and kidney function, ultrasound scan. Based on the received results, the doctor will elaborate the chemotherapy regimen. If necessary, related medical specialists will be involved in the elaboration of a treatment regimen (tumor board).

Chemotherapy is carried out as the inpatient procedure, with mandatory admission to the hospital. After the placement of a venous catheter, you will stay in a comfortable ward. An infusion system will be connected to the catheter, through which the required drug or a drug combination will be administered. All drugs are administered by intravenous drip, slowly, so the total duration of the infusion can be up to several hours. All this time, doctors and nurses will monitor your health condition closely.

After the course of chemotherapy, you will stay under medical supervision in the ward for a few more hours. After the completion of the chemotherapy course you will receive the medical report with detailed recommendations regarding further treatment. In the future, you will be able to have a distant consultation with your attending physician and schedule the next course of chemotherapy, if necessary.

Required documents

• Medical records
• MRI/CT scan (not older than 3 months)
• Biopsy results (if available)

Type of program : Inpatient

Duration: 7 days

Surgical intervention (Limb salvage surgery) 

The program includes:

• Initial presentation in the clinic
• clinical history taking
• physical examination
• review of medical records
• laboratory tests:

• complete blood count
• general urine analysis
• biochemical analysis of blood
• tumor markers
• indicators of inflammation
• indicators blood coagulation

• CT/MRI scan of the local tumor
• CT Chest with contrast
• preoperative care
• tumor resection
• histologically and immunohistochemically examination
  of the remote tissues
• symptomatic treatment
• control examinations
• the cost of essential medicines and materials
• nursing services
• full hospital accommodation
• explanation of future recommendations

Required documents

• Medical records
• MRI/CT scan (not older than 3 months)
• Biopsy results (if available)

Type of program : Inpatient

Expected duration of the program: 10 days

Radiotherapy of Ewing Sarcoma

The program includes:

• Initial presentation in the clinic
• clinical history taking
• review of medical records
• physical examination
• laboratory tests:

• complete blood count
• general urine analysis
• biochemical analysis of blood
• tumor markers

• CT / MRI of local tumor
• full curse of radiotherapy
• nursing services
• consultations of related specialists
• explanation of individual treatment plan

How program is carried out

During the first visit, the doctor will conduct a clinical examination and go through the results of previous laboratory tests and instrumental examinations. After that, you will undergo an additional examination, including complete blood count, laboratory assessment of liver and kidney function. Based on the received results, the physician will conduct radiotherapy planning with the help of CT or MRI, make the permanent tattoo marks on the skin and conduct CT simulation in order to assess the accuracy of the rays and the radiation dose. If necessary, related medical specialists will be involved in the elaboration of a treatment regimen (tumor board).

Radiation therapy is carried out as the day hospital procedure, without mandatory admission to the hospital. At each visit, the physician will assess your general condition and the marks on the skin. After that, you will be placed in a shielded radiation therapy room, on a special table.

Each radiation therapy session lasts less than half an hour (including preparation). All this time, doctors and nurses are monitoring your condition, you can communicate with them through a loudspeaker. The procedure is completely painless. Depending on the planned course of treatment, you will visit the hospital from 1 to 3-5 times a week.

After the completion of the radiation therapy course, you will undergo control examinations aimed at assessing your condition and efficacy of treatment. After that you will receive the medical report with detailed recommendations regarding further follow-up and treatment. In the future, you will be able to have a distant consultation with your attending physician and schedule the next course of treatment, if necessary.

Required documents

• Medical records
• MRI/CT scan (not older than 3 months)
• Biopsy results (if available)

Type of program : Outpatient