Ewing sarcoma, which affects children and young people, is a rare but aggressive bone and soft tissue cancer. It usually develops in long bones or soft tissues such as pelvis, rib cage, ribs. This disease was named after the American pathologist in 1921. It is from James Ewing and is a part of a group of cancer, known as Ewing Sarkom Family Tumors (ESFTS). Ewing sarcoma is a type of tumor with a rapid growing and high spread potential. The disease begins with the rapid proliferation of abnormal cells in or around the bones. Over time, it can metastasize to the nearby healthy tissues and even the lungs, bone marrow and other organs. Although it is usually seen in children and young people between the ages of 10-20, it can rarely be diagnosed in adults.
What is ewing sarcoma?
It is a rare but aggressive type of cancer that affects bone and soft tissues. This disease, usually seen in children, adolescents and young adults, is characterized by tumors that grow rapidly and have the potential to spread to other parts of the body. Most often occurs in long bones (thighs, tips, arm bone), pelvis, rib cage and ribs. However, it may rarely develop in muscles, fat tissue or other soft tissues. This type of cancer occurs when the cells in or surrounding the bones are uncontrolled. Ewing sarcoma is a rapid moving tumor, as early diagnosis and treatment are of great importance. The disease is usually manifested by bone pain, swelling, limitation of movement and fragile bone structure. Since it is highly likely to make metastasis, the lungs can spread to bone marrow and other organs.
The exact cause of Ewing sarcoma is not known exactly. However, research shows that it is associated with chromosomal translocations (genetic changes) in the EWSR1 gene. It is known that the disease is not hereditary, that is, it does not directly pass from parents to children. However, these genetic mutations can affect cell growth mechanisms and cause cancer formation. Since the ewing sarcoma is an aggressive tumor, it is of great importance to start treatment early. Standard treatment approaches include chemotherapy, surgical intervention and radiotherapy. In recent years, new methods such as targeted treatments and immunotherapy have been carried out. Successful results can be obtained in a significant portion of patients with early diagnosis and appropriate treatment.
What are the symptoms of ewing sarcoma?
Since it is a bone and soft tissue cancer with a rapidly growing and high spread potential, its symptoms may not be evident in the early period. In addition, symptoms can be mixed with other musculoskeletal diseases. The disease usually begins with symptoms such as pain, swelling and limitation of movement in bones or soft tissues. In particular, bone pain, especially at night, is one of the early symptoms of the disease. Pain may be exacerbated over time because the tumor grows to weaken bone tissue and pressure on the surrounding nerve tissues.
Ewing sarcoma may rarely develop in ribs, spine and even soft tissues. As the tumor grows, the patient can cause difficulties in daily activities. In the following stages, bone fractures, shortness of breath (if spread to the lung) and weight loss such as general cancer symptoms may occur. When the disease begins to spread to the body, systemic symptoms such as weakness, loss of appetite and fever may also occur. Since the ewing sarcoma tends to make metastasis, early symptoms should not be ignored and it is important that people with long -term pain in bone or muscles are important to consult a doctor. Early diagnosis may increase the chances of healing patients by increasing treatment success. The ewing sarcoma is as follows:
- Pain in the bone and its surroundings: Especially pain is the most common symptom that increases at night and does not go to relax. Pain may increase with physical activities.
- Swelling and mass formation: In the affected area, there may be visible or hand -felt swelling. Swelling is usually hard and painful.
- Movement limitation: If the tumor is located near the joint, there may be a loss of hardness and flexibility in movements.
- Bone fractures: Since the tumor weakens the bone, easy breaks may be seen even in mild traumas.
- General Cancer Symptoms: Unsurable weakness, weight loss and anorexia may be. In advanced stages, body resistance decreases and predisposition to infections may increase.
- Fire and Night Sweats: Intermittent fever and night sweats may be seen due to inflammation (inflammation) and reaction of the immune system.
- Respiratory Problems: If the tumor is spread to the lungs, symptoms such as shortness of breath, chest pain and cough may occur.
- Specific symptoms connected to the settlement of the tumor: If there is a spine involvement, the loss of sensation due to back pain and nerve compression may develop. If there is a tumor in the Pelvis region, there may be problems in walking and urinating problems.
How is Ewing Sarcoma diagnosed?
The diagnostic process is performed by the application of advanced tests such as evaluation of the patient’s symptoms, physical examination, imaging methods and biopsy. First, a detailed physical examination is performed when the patient consults with symptoms such as bone pain, swelling and limitation of movement. If there is a suspicious condition, the doctor uses various imaging methods to see the progress of the disease and the changes in the bones. Methods such as X-ray (X-ray), Magnetic resonance imaging (MR) and computed tomography (CT) provide information about the location, size and spread of environmental tissues. Positron emission tomography (pet scanning) and bone scintigraphy are used to determine whether the disease is spread to other parts of the body. However, a biopsy must be made to make a definite diagnosis. During biopsy, the existence of cancer cells is investigated by examining the tissue sample taken from the suspicious area in the laboratory.
Molecular genetic tests also play an important role in the diagnostic process. Ewing sarcoma is associated with chromosomal translocations in the Ewsr1 gene. Therefore, the disease -specific genetic changes can be detected by using fluorescent in situ hybridization (FISH) or polymerase chain reaction (PCR) tests. Blood tests (full blood count, inflammation markers and liver function tests) can also be applied to determine the degree of spread of the disease. By bringing together all these diagnostic methods, the most appropriate treatment plan is created by evaluating the stage of the Ewing sarcoma, the spread status and the general health status of the patient.
What is the treatment of ewing sarcoma?
The treatment process is usually carried out with a versatile approach, including chemotherapy, surgical intervention and radiotherapy. The age of the patient, the location of the tumor, the spread status is the most important factors that determine the ewing sarcoma treatment options. The following methods are used in treatment options:
- Chemotherapy: The first step in the treatment of ewing sarcoma is usually chemotherapy. Because this tumor tends to grow rapidly and has the potential to spread to different parts of the body even at an early stage. Chemotherapy is applied to reduce tumor cells, control metastases and reduce the tumor before surgical intervention.
- Surgical intervention: It can be surgically removed when the tumor is sufficiently reduced. The aim of the surgery is to completely clean the tumor and prevent it from spreading to healthy tissues. However, if the tumor is located in a critical part of the bones, reconstructive surgery or prosthesis applications may be required to preserve the function of the patient after surgery.
- Radiotherapy: Radiotherapy can be applied if it is not possible to completely remove the tumor or if the tumor residues remain after surgical.
In recent years, immunotherapy and targeted treatment methods are also being investigated. These treatments have been developed to make cancer cells more visible to the immune system or to stop tumor growth by targeting genetic mutations. In addition, supportive treatments can be applied to reduce the side effects of chemotherapy and strengthen the immune system. Ewing sarcoma treatment is usually a complex process that includes many different stages. Patients should be monitored regularly after treatment and care should be taken against possible recurrence.
