Ewing's Sarcoma: Symptoms and Treatment

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Posted on : Dec 22, 2022

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Ewing’s sarcoma is a term used to describe cancer that mostly affects soft tissue or bone. James Ewing, a physician who initially reported the tumour in the 1920s, is the source of the name. The hip bones, ribs and long bones, such as the femur (thighbone), tibia (shinbone) and humerus (upper arm bone), are where this type of cancer most frequently develops. It may also affect the surrounding soft tissues and muscles. The bone marrow, lungs, kidneys, heart, adrenal glands and other soft tissues are only a few of the body parts where its cells can metastasise or spread. Ewing’s sarcoma cells look small, round and blue under a microscope.

This type of cancer makes up about 1% of all childhood cancers and is the second most frequent kind of bone cancer in kids and young people. It has an annual incidence of about 2–3 cases per million people. 

Ewing’s sarcoma can appear at any point throughout childhood, although it most frequently appears around puberty when the bones are quickly expanding. Most frequently, youngsters between 10 and 20 years of age develop Ewing’s sarcoma. Men are more commonly affected than women. Children of African-American, African and Chinese descent rarely develop this kind of cancer. Adult Ewing’s sarcoma is an extremely uncommon malignancy.

Causes

It is unclear what causes Ewing’s sarcoma exactly. Researchers have not been able to identify its risk factors or treatment options. However, scientists have found that chromosomal alterations in a cell's DNA can result in its development. No one genetically inherits these changes. After birth, they appear to develop in youngsters for no apparent cause.

The majority of the time, chromosomes 11 and 22 are fused to create the alterations. The EWS gene on chromosome 22 is 'switched on' when a specific segment of chromosome 11 is inserted adjacent to it. When cells grow too much as a result of this activation, cancer eventually develops. Less frequently, chromosome 22 and another chromosome exchange DNA, resulting in the activation of the EWS gene. Although the precise process is yet unknown, this significant finding has improved the ability to diagnose Ewing’s sarcoma.

Symptoms 

The symptoms that Ewing’s sarcoma patients experience can vary. The following are among the most typical signs:

  • Pain at the tumour's location
  • Redness or swelling surrounding the tumour's site
  • Fever
  • Loss of weight and reduced appetite
  • Persistent tiredness
  • Either a paralysis or incontinence, especially when the tumour is in the spinal region
  • Symptoms brought on by the tumour's nerve compression, such as numbness, tingling or paralysis

Ewing’s sarcoma symptoms may resemble those of other illnesses or issues. Always seek a diagnosis from your doctor.

Diagnosis 

Diagnostic tests assist in confirming the presence of a tumour and also provide information about the tumour that can help oncologists decide on the best course of treatment, in addition to a comprehensive medical history and physical examination. The following are examples of possible diagnostic tests:

Multiple imaging tests:

X-ray

This diagnostic procedure creates images of interior organs, bones and tissues on film using invisible electromagnetic energy beams.

Radionuclide bone scan

A small quantity of radioactive dye is first injected into the patient's bloodstream, where the bone tissue absorbs it. With the aid of a specialised camera, images of the bone are taken in order to look for malignancies and other bone abnormalities.

Magnetic resonance imaging (MRI)

This diagnostic test creates precise images of the body's organs and structures using a combination of massive magnets, radio frequencies and a computer. This examination is frequently performed to check for tumour metastasis to the adjacent soft tissues.

Computed tomography (CT or CAT) scan

In this diagnostic imaging process, horizontal or axial images of the body—often referred to as slices—are created using a combination of X-rays and computer technologies. Any aspect of the body, including the bones, muscles, fat and organs, can be seen in great detail on a CT scan. MRI scans typically have greater detail than CT scans, which are typically less detailed.

Blood tests 

These include blood chemistries.

Positron emission tomography

Glucose (sugar) with a radioactive tag is injected into the patient's bloodstream for this test. A scanning device can identify tissues (such as cancers) that utilise glucose more intensively than typical tissues. They could also be used to  detect tiny cancers or to determine whether a recognised tumour is responding to therapy.

Biopsy of the tumour

In this process, tissue samples are taken from the body (either surgically or with a needle) and analysed under a microscope to check for the presence of cancer or other atypical cells.

Bone marrow biopsy or aspiration.

In order to determine whether cancer has progressed to the bone marrow, this operation entails removing a miniscule bit of bone marrow fluid and tissue, typically from a portion of the hip bone.

Treatment

Chemotherapy

An oncology team will take over patient care after the cancer stage has been established and will administer chemotherapy. To decrease the size of the primary tumour, chemotherapy is necessary for all Ewing’s sarcoma patients. It  is always the initial step in treatment, regardless of other factors, such as how the tumour is excised or dealt with locally. This is because Ewing’s sarcoma is treated as both a local and systemic disease by clinicians. The somewhat common but highly treatable metastasis to the lungs is treated with chemotherapy. Even when the malady seems to be localised at diagnosis, a multimodality strategy is applied.

Radiation or Surgery to Treat the Tumour

Patients undergo a second MRI and CT scan of the chest after the initial chemotherapy to restage the tumour.

The patient will often undergo a resection if the tumour is operable. Typically, surgery is advised as an alternative to radiation, which can have serious adverse effects, especially in young children, if cancer can be excised.

Radiation may be necessary if the tumour cannot be removed surgically. Radiation therapy and surgery are both sometimes necessary.

Additional Chemotherapy

Following surgery or radiation therapy, a second round of chemotherapy is administered to eradicate any tumour cells that may have metastasis to other regions of the body.

Targeted Therapies

Finding a treatment that is already approved by the Food and Drug Administration for cancers with specific biomarkers, which are characteristics that may indicate that a tumour is a good target for a specific therapy, is possible using genomic sequencing.  Even medications that don't have a prior record of being used to treat Ewing's sarcoma might well be taken into consideration.

There might potentially be clinical studies available. Your doctor can notify you of any ongoing studies at your local cancer centre or at other facilities across the nation. Aster is the best cancer hospital in Bangalore, offering the best medical care to ensure complete health and well-being. 

FAQs

Which bones are most likely to be affected by Ewing’s sarcoma?
Predominantly, the long bones, such as the femur, fibula, tibia and the pelvis bone, account for more than 50% of all cases. 

Are children given the same treatment as adults?
Yes, chemotherapy remains the same for both. However, it has been found that children handle chemotherapy better than adults. 

Can you genetically inherit Ewing’s sarcoma from your parents?
No, it is not possible to inherit Ewing’s sarcoma.

What is the annual incidence of Ewing’s sarcoma?
Ewing’s sarcoma presents itself in 2–3 cases per million in population. 

How is Ewing’s sarcoma treated?
It is generally treated with chemotherapy, surgery and radiotherapy.

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