Ewing’s Sarcoma – Prognosis, Survival Rate, Symptoms, Treatment


What is Ewing’s Sarcoma?

Ewing’s Sarcoma is a cancerous tumour in the bone that primarily affects children. It is a very rare form of bone cancer that affects adolescents and young children. Ewing’s Sarcoma is caused by an alteration in the chromosomal arrangement of the child after birth.

There are no known reason why the chromosomes are altered, there are also no definite risk factors associated with Ewing’s Sarcoma. This condition is rare that is why we have few studies to use and the data are only able to describe the characteristics of Ewing’s Sarcoma.

Ewing's Sarcoma


Ewing’s Sarcoma on the Right Femur

Symptoms of Ewing’s Sarcoma

The symptoms of Ewing’s Sarcoma is easy to note. However, they are very general and it may be related to other conditions. In some cases, a fracture can happen just after a minor injury. I have listed the symptoms that may indicate that a child has Ewing’s Sarcoma.


  • Pathologic Fracture –  After a minor injury, a child can have a broken bone. This is a symptom that is very common to Ewing’s Sarcoma patients.
Ewing's Sarcoma Fracture
Ewing’s Sarcoma with Signs of Small Fractures
  • Fever –  Although this is a general defense mechanism of our body against systemic infection and other foreign bodies, this can be present in a patient with Ewing’s Sarcoma. Please note that this can also be associated with other disease that is why fever is not a definitive symptom of Ewing’s Sarcoma.
  • Pain – Because a tumor is growing out of the bone, there will be pain present. This is not a definitive symptom because this can also be a pain from a broken bone or any other causes.
  • Swelling – This is also a general defense mechanism of our body to fight against a local infection. This can be present to a bone fracture or any other causes.

Causes of Ewing’s Sarcoma

Ewing’s Sarcoma is a rare condition that affects children aged 10 – 20. This is because the bones are growing very fast in this age. The bone tumor may grow anywhere in the body.

Usually, it can be seen at the long bones (arms and legs). It can also develop in our short and flat bones (like skull, pelvis, and scapula). Bones cancer can spread to the lungs and other adjacent organs. Although we have discussed that this condition targets children, it can also appear in adults.


The cause of Ewing’s Sarcoma is not clearly identified, there are no exact and definitive risk factors associated with Ewing’s Sarcoma. I listed the known risk factors in having Ewing’s Sarcoma.

  • History of previous cancers– This is included in our Risk Factors because cancers are can be treated using Radiotherapy, and Radiotherapy can cause a mutation of normal cells. This can predispose a person to Ewing’s Sarcoma by the bone’s exposure to radiation.
  • Presence of an inborn disease– This can cause a change in chromosomal structure in children, especially the newborn.

Diagnosis of Ewing’s Sarcoma

To fully diagnose Ewing’s Sarcoma, we need to have a series of tests done to the patient. I will list down the diagnostic tests and exams here.

  • X-ray – An x-ray will be ordered to clear out the suspicion of Ewing’s Sarcoma. This procedure is done with other tumors to check its origin (bone or other tissue). Please note that this procedure will only give a minimum exposure to radiation, this will not likely be the cause/aggravate the status of the tumor.
  • Advanced Imaging Tests – This includes MRI and CT Scan, they are both specialized imaging test that is used to scan the tumor deeper. It will produce a detailed result on the status of the tumor.
  • Biopsy – Bone Biopsy will be used to extract specimens from the tumor. This is to ensure an accurate diagnosis because there are other types of cancers that can affect the bones, and the patient may have a previous cancer that may have reached the bones.

Treatment of Ewing’s Sarcoma

Children is the most common victim of Ewing’s Sarcoma, using that statement, we will need to use chemotherapy as a last resort treatment regimen. Chemotherapy is a treatment regimen that halts the growth and multiplication of the cancer cells, but this therapy will also halt the growth and multiplication of all cells in our body. In that case, if we use a chemotherapy, this will stunt the growth and development process of the child. Although that is a fact, this logic is not followed by doctors because they believe that we should intervene with Ewing’s Sarcoma immediately. This conflict caused a controversy whether to choose a safer treatment or use chemotherapy.


Radiation Therapy

This therapy is used to kill cancer cells. Although radiation can cause cancer. It can also kill cancer cells by having the correct amount of radiation pointed to the location of the cancer cells.

Surgery

This is the most preferred method because this will remove the tumor out of our body.

Chemotherapy

Since Ewing’s Sarcoma is a type of cancer, we can give the patient chemotherapy sessions. This method will kill the cancer cells; however, this can also kill our normal cells in the body.

A combined therapy is developed and it produced an opportunity to cure this type of cancer. The combined therapy is a combination of chemotherapy and surgery (radiation therapy can be also used too). Also, bone marrow transplant can be the future treatment to treat and prevent this type of cancer.

Prognosis and Survival Rate

Patients with Ewing’s sarcoma has an overall 5-year survival rate of 66%. A good prognosis can be decided early in the treatment phase when the initial chemotherapy sessions produced a good response from the tumor. Patients who have tumors that metastasized have lower prognosis than patients who do not. However, patients who have their tumor metastasized to the lungs has a greater prognosis than those who have their tumors metastasized to the bone.

These are the factors that will affect the prognosis of children who have Ewing’s sarcoma:

  • Age – this will affect the treatment tolerance of the patient, some researchers say that a young patient has a low tolerance to cancer treatment.
  • Size of tumor – this is to know if the tumor is metastasized to other adjacent body organs.
  • Tumor’s Response to Therapy – this is to know if the current treatment regimen is working and this is to evaluate the treatment’s efficiency.
  • Location of the tumor – this will affect the prognosis of the child because the location of the tumor will affect the treatment modality to be used and it can also affect the treatment’s efficiency if the tumor is superficial or deep within.

Staging of Ewing’s Sarcoma

Primary Tumor

  • T1 – 8 cm or less in greatest dimension
  • T2 – >8 cm
  • T3 – discontinuous tumors in the primary bone site

Regional Lymph Nodes

  • N0 – no
  • N1 – yes

Distant Metastases

  • M0 – no
  • M1a – lung
  • M1b – other distant sites

Stage Grouping

  • IA – T1 N0, Low grade
  • IB – T2 N0, Low grade; or T3 N0, Low grade
  • IIA – T1 N0, High grade
  • IIB – T2 N0, High grade
  • III – T3 N0, High grade
  • IVA – M1a
  • IVB – N1, M1b

Note: Ewing’s sarcoma is classified as grade 4.

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