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August 12, 2020

Ewing Sarcoma – Symptoms and Treatment

About Ewing Sarcoma and its Symptoms

Ewing’s sarcoma is a very rare tumor of bone and soft tissue. It is affecting two million children in the United States. The peak age at which we observe Ewing Sarcoma is usually in the teenage years or the early twenties. Although Ewing sarcoma symptoms develop in childhood also. When the tumor arises in teenagers it often presents as pain or swelling around a joint or at another large muscle or bone in the body.

People initially thought it to be a sprain or a sports injury because these tumors tend to arise in teenagers and patients in their twenties. Many patients are athletic and might pass it off initially just as being a sports injury.

When the pain or swelling doesn’t resolve within about a week or so with conservative measures or it’s clear that it is swelling that’s more than just a sprain that will generally tell the physician and patient that it is something more serious.

What are the types of Ewing’s sarcoma?

As per medical professionals, the types of Ewing’s sarcoma classified into three types.

  • Localized Ewing’s sarcoma
  • Metastatic Ewing’s sarcoma
  • Recurrent Ewing’s sarcoma

Let’s discuss the above types in detail:

Localized Ewing’s sarcoma

In this type, a patient has developed cancer but it hasn’t spread to the other sites of the body.

Metastatic Ewing’s sarcoma

In this type, cancer started to spread other parts of the body and it includes lungs.

Recurrent Ewing’s sarcoma

This is the most dangerous type of Ewing’s sarcoma as your body doesn’t respond to the treatment given by the pediatric or medical oncologist, and it results in Recurrent Ewing’s sarcoma.

What are the Symptoms of Ewing sarcoma?

People who are suffering from Ewing’s sarcoma may present pain or swell around a joint or at another large muscle or bone in the body. Poor appetite, a high temperature of the body, sudden changes in weight, exhaustiveness, low levels of energy, anemia, and a bone that breaks without a specific reason are the symptoms associated with Ewing’s sarcoma.

Ewing’s sarcoma forms typically in the following body parts. It includes

  • Arms
  • Legs
  • Pelvis
  • Chest

Some patients with Ewing’s sarcoma may experience difficulty in breathing or unable to breathe if the tumor is present in the chest.

When to consult a physician

Consult your doctor or a healthcare provider immediately if you are experiencing any of the symptoms of ewing sarcoma. Your doctor should evaluate you for other possible causes of your symptoms before treating Ewing sarcoma. Together, you and your doctor can determine the best way to manage your symptoms.

What are the causes of Ewing’s sarcoma?

Researchers are yet to find the exact cause of this condition. People might think that the main cause of Ewing’s sarcoma can be genes but it is not true. There is no source to this point. As excess of healthy cells die and new cells developed in your body. Some cells cannot form in a good manner and it results in the formation of a tumor and it will destroy the remaining healthy cells in the body.

When it comes to Ewing’s sarcoma, EWSR1 is a gene affected by the DNA that can contribute to this condition. Due to this reason, your physician will test this gene if you are showing the signs and symptoms of Ewing sarcoma.

What are the risk factors of Ewing’s sarcoma?

Several well-established risk factors developing this condition. It includes

  • Age
  • Ancestry

Let’s discuss the above risk factors in detail

Age

This condition can affect people of all ages but it occurs mostly in children and young adults. They have an increased risk of diagnosing with Ewing’s sarcoma. Research shows that fifty percent of the people with the condition diagnosed in adolescence.

Ancestry

People who belong to the European ancestry are more likely to develop Ewing’s sarcoma. This condition is far less common in people who belong to the African and East Asian ancestry.

Gender

Females are far less likely to develop this condition than males. Research shows that fifty-nine percent of the males were affected by Ewing’s sarcoma in the study conducted to 1426 people and the rest are females.

What are the complications of Ewing’s sarcoma?

Difficulty in breathing or unable to breathe, weakness or paralysis of muscles, and pleural effusion are the complications associated with Ewing’s sarcoma. It is really for the people who are suffering from these complications to see a physician. Your doctor can prescribe medication to give you relief from those complications.

This condition can spread from where it started and due to this reason, your physician will do a complete evaluation to determine if the tumor has spread to other sites of the body.

How do physicians diagnose Ewing’s sarcoma?

The approach to a patient with Ewing sarcoma is to make the diagnosis and to do the complete evaluation of the patient to determine if the tumor has metastasized or spread to parts outside of where the primary tumor originated. Diagnosing this condition involves getting a biopsy of the tumor which is either done surgically or via a Computed tomography scan or ultrasound-guided biopsy to get a piece of the tumor to look at the tumor under the microscope to find the symptoms of ewing sarcoma and confirm the diagnosis.

Once the diagnosis confirmed then the patient needs to undergo a complete evaluation which includes a chest CT scan and an MRI of the involved area where the primary tumor is. Your physician may recommend positron emission tomography to determine the sites of the spread of the tumor as well as response to treatment.

An evaluation of the bone marrow and it is due to this condition can also spread to the bone marrow not just to other bones. The initial evaluation of a patient with Ewing’s sarcoma as well as the biopsy should perform at a center that is experienced in managing such a rare tumor.

How is Ewing’s sarcoma treated?

The treatment of Ewing sarcoma is truly multidisciplinary and the treatment done by a pediatric or medical oncologist in conjunction with the surgeon and radiation oncologist. It will ultimately involve treatment with chemotherapy as well as treatment with surgery and radiation therapy.

Depending on the site of the tumor and depending on the response of the tumor to chemotherapy will ultimately decide your physician perform surgery. Radiation therapy used to perform to control the Ewing sarcoma followed by the additional therapy.

The standard treatment is currently a combination of drugs called vincristine, doxorubicin, which is the same thing as Adriamycin, and cyclophosphamide given every two weeks can improve the outcome of the treatment done through chemotherapy followed by surgery.

The treatment and cure rate of healing sarcoma has improved significantly over the past twenty years through the incorporation of the results of the clinical trials.

Conclusion

If you are experiencing the symptoms that you think is Ewing sarcoma then you should talk with your physician about your symptoms.

Your doctor can complete a physical exam and review your medical history to determine which treatment option would be best for you. It is important to get an accurate diagnosis and prompt treatment.

References

  1. https://pubmed.ncbi.nlm.nih.gov/27297500/
  2. https://acsjournals.onlinelibrary.wiley.com/doi/epdf/10.3322/canjclin.22.2.95
  3. https://www.hindawi.com/journals/sarcoma/2011/863210/
  4. https://onlinelibrary.wiley.com/doi/abs/10.1002/pbc.20095?
  5. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3164520/
  6. https://www.nature.com/articles/s41572-018-0003-x

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