Ewing Sarcoma Diagnosis

Ewing Sarcoma Diagnosis and Treatment

Makaylah Mutz

Biology 1501

Professor Benard

April 19, 2018

Introduction

Ewing Sarcoma is a form of cancer that starts growing as tumors in bones and close to the soft tissues that are grouped near bones. Any cells in the body can become cancerous and then can move throughout the body. Any form of cancer begins by the rapid division of cells and that end up growing out of control crowding out the normal body cells. Ewing Sarcoma tends to have gene of DNA the fuses into abnormalities and consist of proteins that are not found or are rarely found within other forms and types of cancers. Ewing Sarcoma is generally found in three main areas of the body: the legs - more specifically

at the center of the long bones, the wall of the chest which includes the shoulder blades and the rib cage and lastly, they are also found to grow on the pelvis area. This form of cancer is known to be very rare. Approximately 1% of all childhood tumors end up being Ewing tumors. These types of tumors are more likely to be found in children and teenagers, however Ewing tumors can also be found in adults (between the ages of 20 and 30). About 225 cases of Ewing tumors a year in North America in children and teens. Approximately 75%-85% of people who are diagnosed with Ewing sarcoma regardless of aggressive chemotherapy, radiation and surgery die within a span of 5 years or so (Winter). Out of the cases that involve Ewing tumors, more males than females end up developing this form of cancer and this was found through investigations about what factors can cause these types of Ewing tumors (Ashour). People of certain races are also more likely to end up with an Ewing’s tumor than another. This disease is more commonly found among whites.
Ewing Sarcoma of bone is found in a family of tumors called the Ewing family of tumors and is the second most common bone cancer in adolescents (Scientific Research Publishing). In this family there are three different forms of tumors, including the Ewing Sarcoma of bone, that are all similar in their abnormalities and proteins. The other two types of tumors in this family are: Extraosseous Ewing tumor (EOE) which grows in the soft tissues close to the bones and the cells look very similar to the Ewing Sarcoma of bone. The next is called Peripheral primitive neuroectodermal tumor (PPNET) which begins growing in soft tissues or bone and closely resembles some features of Ewing Sarcoma of bone and Extraosseous Ewing tumor. This form however is a rare childhood cancer. When comparing these three Ewing tumors some similarities are found. The same proteins and gene abnormalities are found in all three of these types of tumors and not found in other types of cancer. All three forms seem to develop from the same types of cells within the body (American Cancer Society).
With any form of cancer there are risk factors that can make it easier for a person to develop that form of cancer. With Ewing tumors there are some risks, such as: diet, a person’s physical activity level, body weight and in some cases tobacco use can be a large factor for cancer development in adults. Age is also a large risk factor when talking about Ewing tumors. The more common ages for these tumors to develop is during an individual’s teen years. The chances of developing Ewing tumors very young or in an individual’s young adult years and in older adults is rarer. Gender is another risk factor when talking about Ewing tumors. Ewing tumors can happen to any one person at any given time, but the development of an Ewing tumor has been found to be more common in males than in females, the reasons as to why this is the case is not known. Ethnicity plays a role in what person may end up developing a tumor. Ewing tumors tend to be more commonly found among whites (Hispanics or non-Hispanics). Ewing tumors are less likely to be found in Asian Americans and very rare in African Americans. With all of these risk factors in mind, it will usually take many years for any of these risks to influence the development of cancer and these risks are also not found to have any type of influence on childhood cancer, including Ewing tumors. It was also found that Ewing tumors aren’t inherited, which means formations of tumors can happen to anyone and just because a family member had a Ewing tumor does not mean a member of the same family will inherit a gene for Ewing tumors (Surgery). The gene mutations that are responsible for the development of Ewing Tumors are EWSR1 which is located on the twenty-second chromosome and FLI1 which is located on the eleventh chromosome. When these two genes fuse together they create the abnormal gene that creates Ewing tumors (Ewing sarcoma).
EWSR1 and FLI1
Approximately 85% of all Ewing sarcoma cases are caused by the translocation of the fusion of EWSR1 and FLI1. The fusion of these two genes creates a gene-product EWS-FLI1. Within this gene-product each gene that was fused contributes a crucial role to the development and growth of cells. The gene ESW has a transcriptional component and FLI1 has a DNA binding component. The combination of both of these genes act as a transcriptional repressor (Winter).
Diagnosis
When a doctor or patient is suspicious about possibly having Ewing sarcoma, the first step to the diagnosis is to have a physical examination.

During the physical examination the doctor will be looking for any areas that are causing the patient pain and areas that might have some swelling. When pain or swelling is found there are other test that can be run, such as: X-rays, Magnetic resonance imaging scan (MRI), Computed tomography scan (CAT scan), bone scan and a Positron emission tomography scan (PET scan). These scans are performed for the doctors to get a better look at the area in question and to determine the extent of the tumor. If the images from the scans suggest that there is a tumor and that it might be an Ewing tumor a biopsy of the tumor will be ordered. A biopsy of the tumor in question will help to determine if the tumor is either an Ewing’s tumor or some other cancer or that the tumor is a benign tumor which then would mean that the mass of cells is not cancerous. Most biopsies are taken by extracting a sample of the tumor (Scientific Research Publishing) and the later viewing the samples taken under a microscope. Sometimes if the tumor is in a good location and is small the surgeon can remove it completely and observe the entire tumor. Once a diagnosis has been made tests such as a bone marrow aspiration and biopsy test and blood test are done so that doctors can track the spread of the cancer to the bone marrow

(Surgery).
Treatment
When treating Ewing sarcoma chemotherapy is mostly used as the first step of treatment for patients. Chemotherapy also known as chemo is administered through a vein using an IV. The use of this form of therapy is used as an anti-cancer medication that enters the bloodstream and interreacts with the cancer cells which may be likely to spread. When a patient is receiving chemotherapy they normally get it for a minimum of 12 weeks before any other type of treatment. On average a total of 14 – 15 cycles of chemo happen within a span of 6 months to a year. However, with this treatment there are side effects. Some of the side effects are: hair loss, mouth sores, loss of appetite, nausea and vomiting, increased risk of infections, easy bruising and fatigue. Most of these side effects come from the depletion of the amounts of white and red blood cells and having not enough blood platelets (Surgery). When the tumor at the time of diagnosis is a metastatic tumor about 80% of patients die due to the fast progression of the cancer either in stages or throughout the body (Kikuta).
After chemotherapy, surgery is suggested to most patients if the tumor (s) are located in a place where they can be removed. The point of surgically removing the tumors is to completely get rid of all the cancerous cells within the body at that location and make sure that no cancerous cells are left behind to multiply and become a brand-new tumor. Along with removing the tumor surgeons in most cases remove some of the normal tissue around the location of where the tumor was taken from to test the margins and see if they are cancerous or not. The margins are said to be positive if cancerous cells can be seen in the tissue and a negative reading means that all of the cancerous tissue has been removed.
For some patients’ radiation therapy is another treatment option.

When receiving radiation therapy high-energy beams are pointed in the location of the tumor (s). This treatment method can be used instead of surgery or coupled with surgery depending on the complexity of removing the cancerous tumor. When receiving this form of treatment, it is in most cases given about 5 days on average a week for a certain number of weeks. With each treatment of radiation, the patient will be gradually receiving a higher dose of high-energy beams then in the previous radiation therapy session. Radiation therapy itself only takes a couple minutes but getting the radiation machine and the patient in place for treatment may take a much longer amount of time then the actual treatment. With any type of cancer treatment there are going to be some side effects. Some short-term side effects are patches on the skin that look like sun burn and hair loss also in the area where the radiation was aimed. Long-term effects are organ function depending on where the treatment was given and in some cases nerve damage and can also cause headaches and trouble thinking. A major concern with this form of Ewing tumor treatment is the fact that a new form of cancer can develop in the area where the patient had once received radiation

therapy.
The last option is for patients with Ewing tumors that have no chance in being cured with any other treatment or their tumors come back after basic treatment. This treatment is called High-dose chemotherapy and Stem cell transplant, also known as a bone marrow transplant.

Conclusion