What Is Sarcoma?
Cancer conditions are named differently depending on the place of occurrence in the body. Cancer that can occur on the bone and muscles are known as sarcoma. If the sarcoma occurs on the soft tissues like nerves, blood vessels, muscles, and fat, then it is mentioned as soft tissue sarcoma. More than 70 types of sarcoma have been recorded, and each one has a different presentation and mode of treatment. Among them, angiosarcoma, chondrosarcoma, and liposarcoma are the common ones.
What Is Ewing Sarcoma?
Ewing sarcoma is a type of bone cancer that has a very rare occurrence. It is known to affect younger children and teenagers, especially in the first decade of life. The cancer is generally known to start from the long bones of the body and the pelvic bones. The classical site of occurrence is diaphysis of long bones. However, it has the capacity to start from any part of the bone. In cases where soft tissue involvement is seen, it begins in the abdomen, chest, and limbs. Both males and females are known to be affected by Ewing sarcoma. The other names of Ewing sarcoma are round cell sarcoma or PNET (peripheral neuroectodermal tumor). It is possible to cure the patients with Ewing sarcoma, but long-term monitoring is required.
What Are the Types of Ewing Sarcoma?
There are three types of Ewing sarcoma. They are:
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Bone Tumor: The majority of the Ewing sarcoma is known to happen in the bones. This specifically includes bones of the thigh, ribs, pelvis, and shoulders.
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Extra-Osseous Tumor: It is also known as a soft tissue tumor. It can involve all the muscles and cartilage. Older people are known to be affected when compared to other types. The commonest occurrence happens in the trunk region. This type is known to affect both genders equally.
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Askin Tumor: It is a rare type of cancer occurring in the soft tissues. It is known to be a type of primitive neuroectodermal tumor that can occur in the chest region.
What Are the Causes of Ewing Sarcoma?
The actual cause for Ewing sarcoma is not identified. Some genetic mutations have contributed to the sarcoma condition. The prime functioning of the cell depends on the DNA (deoxyribonucleic acid) material. It sends a command to the other cells for regulating the functions. If there is any miscommunication due to the mutations, it might result in genetic problems and abnormal cell growth. These abnormal cells begin to spread and cause the destruction of healthy cells. In Ewing sarcoma, there is an alteration in the gene EWSR1. People who are having severe exposure to radiation are also known to be affected by Ewing sarcoma.
What Are the Symptoms of Ewing Sarcoma?
Patients who are having Ewing sarcoma might experience pain in the involved region. It might be seen along with stiffness in that area. An important characteristic of cancer is pain in the affected region that has been lasting for a prolonged duration of time. The other symptoms associated with Ewing sarcoma are:
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Weight loss.
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The appearance of a lump on the skin that might feel warm on touch.
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Bone pain that is known to increase by doing exercise. It is also known to aggravate during night time.
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If the jaw is known to have a tumor, then the patient might experience numbness and pain in the facial region. Swelling of the jaw is most commonly seen in such cases.
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If the tumor is occurring near the spine, then there will be a loss of control over the bladder, and the patient might suffer from paralysis.
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Classically the presentation of Ewing sarcoma matches with the presentation of osteomyelitis (infection of bone).
What Are the Risk Factors of Ewing Sarcoma?
The risk factors associated with Ewing sarcoma are:
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European race: People belonging to the European race are known to be affected more than other people. It is necessary to be aware of such conditions.
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Age: Since children (less than 10 years of age) are susceptible to Ewing sarcoma, it is necessary for these people to be aware of it.
How Is Ewing Sarcoma Diagnosed?
Diagnosis requires the following procedures and tests:
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Clinical Examination: The doctor will perform a clinical examination to check the involved site. The doctor will record the redness, swelling, and presence of lumps. Past medical history is an important one that has a lot of medical significance. You have to report to the doctor regarding the past usage of medications and surgical procedures.
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X-rays: X-ray is the most preferred tool to identify the tumor area if it is suspected. You would require recommendations from your doctor to perform an X-ray. If the tumor is present on the long bones, X-ray imaging will show onion skin appearance (though considered classical of Ewing sarcoma but can also be seen in other tumors of bone like osteosarcoma).
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MRI Scan (Magnetic Resonance Imaging Scan): It is a radiographic procedure that uses scanners to generate pictures of the body's organs.
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Bone Scan: Bone scan is a type of imaging technique that helps in detecting several types of infections, injuries, and tumors of the bone.
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Positron Emission Tomography: Positron emission tomography is an imaging technique that involves the usage of a radioactive substance to visualize the physiological processes and metabolic activities. It is rarely used for diagnosis of Ewing sarcoma.
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Biopsy: The doctor will request you for a biopsy specimen. It is collected from the involved site and sent to the laboratory for analysis. The two types of biopsy procedures recommended for Ewing sarcoma are:
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Needle Biopsy: In this procedure, a thin needle is inserted on the skin and guided to reach the tumor. Small pieces of tissues are removed from the tumor with the help of a needle.
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Surgical Biopsy: In this procedure, the doctor makes an incision and takes tissue for diagnosis.
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What Are the Treatment Options for Ewing Sarcoma?
The first-line treatment of Ewing sarcoma is chemotherapy.
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Chemotherapy: It is a treatment that uses powerful medication to treat cancer cells. It is usually combined with other drugs depending on the health condition. The drugs have the capacity to shrink the tumor. Later, doctors can easily remove the tumor from the targeted site. It is done along with radiation therapy for some patients. When chemotherapy is given along with radiation therapy, the rate of prognosis is very good.
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Radiation Therapy: It is a method that uses high energy radiation such as protons and X-rays. They help in destroying cancer cells. If Ewing sarcoma is located in a region that is not easily accessible by surgery, then radiation therapy is the best option. In advanced Ewing sarcoma cases, radiation therapy is known to alleviate pain and slow cancer growth.
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Surgery: A surgery is planned after coordinating with the doctor. The surgical options involve removing the involved part of the bone. In some patients, additionally, a larger tissue is removed to prevent a recurrence. In rare cases, amputation of the limb is required.
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Psychological Counseling: Patients with Ewing sarcoma are known to suffer severe depression. They would require proper psychological counseling to overcome their complicated health conditions.
Conclusion:
Ewing sarcoma is a cancer of the bone that can be completely treated when detected at the early stages. However, long-term observation is needed. In this type of sarcoma, chemotherapy is first done to reduce the tumor size which is then followed by radiation therapy and surgery. Since Ewing sarcoma most commonly affects children below the age of ten years, prolonged pain at a particular site should not be neglected. Reach out to healthcare professionals if there is severe pain for long periods to rule out Ewing sarcoma.
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