Ewing Family of Tumors

Ewing Family of Tumors

Ewing Family of Tumors (EFTs) is a rare type of cancer that affects children and young adults. EFTs are a group of cancers that include Ewing sarcoma, primitive neuroectodermal tumors (PNETs), and Askin tumors. These tumors usually arise in the bones or soft tissues of the body, and they can occur in any part of the body but are most commonly found in the pelvis, thigh bone, and chest wall.

EFTs are characterized by the presence of a specific genetic abnormality, which is a translocation between chromosomes 11 and 22. This results in the fusion of two genes, EWSR1 on chromosome 22 and FLI1 on chromosome 11, which produces a new fusion protein that is thought to be involved in the development of these tumors.

Cause and Risk Factors

The Ewing Family of Tumors causes changes in DNA cells. It causes DNA cells to multiply quickly. It results in the formation of abnormal cells and uncontrolled growth leading to the spread of cancer. It also destroys healthy body tissue affecting the gene called EWSR1. There are several risk factors that can cause the Ewing Family of Tumors such as –

• Age – Children and teens are more likely to get affected by the Ewing Family of tumors compared to adults.
• Gender – More common in males than females.
• Race/Ethnicity – Ewing tumors are rare but they are more common in white individuals than in African/Asian individuals. 

Ewing Family of Tumors

Symptoms

The signs and symptoms of Ewing Family tumors last for a few weeks or months depending on the areas of the tumor such as arms, legs, chest, back, or pelvis. 

• Low fever
• A lump or swollen skin that feels warm and soft
• Broken bones
• Weight loss
• Unexplained tiredness
• Leg pain and limbing

If you notice any of the above symptoms it is necessary to consult a doctor for effective diagnosis and treatment plans. Earlier detection and treatment the better.

Diagnosis and Tests

The Ewing family of tumors (EFTs) is a group of rare malignant tumors that typically affect bones or soft tissues, and most commonly occur in children and young adults. EFTs are diagnosed through a combination of clinical evaluation, imaging studies, and laboratory tests.

1. Clinical evaluation – The first step in diagnosing EFTs is a thorough physical examination, which includes a medical history and a complete physical examination. The doctor will examine the area of concern and may ask questions about the patient’s symptoms, including pain, swelling, and fever.

2. Imaging studies – Imaging studies are essential for the diagnosis of EFTs. These may include X-rays, CT scans, MRI scans, bone scans, and PET scans. These tests help to visualize the tumor and its location, as well as any possible spread to other parts of the body.

• X-rays – It takes images of the area where the tumor is suspected.
• Computed Tomography (CT) scan – In patients with suspected EFTs, a CT scan may be used to visualize the primary tumor and any possible metastases. CT scans can provide detailed images of the bones and soft tissues, allowing doctors to identify the location and size of the tumor.
• Magnetic Resonance Imaging (MRI) – In patients with suspected EFTs, MRI is particularly useful for visualizing soft tissue structures, such as muscles, tendons, and ligaments, and for identifying the extent of the tumor in relation to nearby structures. MRI can also provide information about the tumor’s size, shape, and location, as well as any possible spread to nearby lymph nodes.
• Bone scan – During a bone scan, a radioactive tracer is injected into a vein in the arm. The tracer travels through the bloodstream and is taken up by the bones. The patient then lies down on a table while a special camera takes pictures of the bones. The radioactive tracer emits gamma rays, which are detected by the camera and used to create images of the bones. In patients with EFTs, a bone scan can help to determine whether cancer has spread to other bones in the body. 
• Positron Emission Tomography (PET) scan – In patients with suspected EFTs, a PET scan can help to identify areas of the body with high metabolic activity, which may indicate the presence of cancer. PET scans can also help to distinguish between active tumor tissue and scar tissue or necrotic tissue, which can be helpful in determining the extent of the tumor.

3. Biopsy – A biopsy is the definitive diagnostic test for EFTs. During a biopsy, a small sample of the tumor is removed and examined under a microscope to determine whether it is cancerous. There are different types of biopsies that can be performed, including needle biopsy, core biopsy, and surgical biopsy.

4. Laboratory tests – Laboratory tests can also be helpful in the diagnosis of EFTs. These may include blood tests, such as a complete blood count (CBC), which can show anemia or elevated white blood cell counts, and tumor marker tests, such as a C-reactive protein (CRP) or lactate dehydrogenase (LDH) test.

Once a diagnosis of EFT is confirmed, additional tests may be done to determine the stage and extent of the disease, as well as to help guide treatment. These tests may include bone marrow biopsy, pulmonary function tests, and other imaging studies, such as a bone scan or a CT scan of the chest, abdomen, and pelvis.

Treatments

The treatment of the Ewing family of tumors (EFTs) typically involves a combination of chemotherapy, surgery, and radiation therapy. The specific treatment plan will depend on the location and extent of the tumor, as well as the patient’s age and overall health.

1. Chemotherapy – is typically the first-line treatment for EFTs. Chemotherapy involves the use of drugs to kill cancer cells and is usually given in cycles over a period of several months. The drugs used in chemotherapy can be given intravenously or orally. Chemotherapy is often used before surgery to shrink the tumor and make it easier to remove, and after surgery to kill any remaining cancer cells.
2. Surgery – is another treatment option for EFTs, particularly for tumors that are located in a single area and have not spread to other parts of the body. The goal of surgery is to remove as much of the tumor as possible while preserving nearby healthy tissue. In some cases, surgery may be combined with radiation therapy to help kill any remaining cancer cells.
3. Radiation therapy – involves the use of high-energy radiation to kill cancer cells. Radiation therapy is typically used after surgery to kill any remaining cancer cells, or in combination with chemotherapy for tumors that cannot be surgically removed. Radiation therapy may also be used to relieve pain or other symptoms caused by the tumor.

In addition, to these standard treatments, clinical trials may be available for patients with EFTs. 

Clinical trials 

Research studies are conducted to test new treatments or combinations of treatments. Participating in a clinical trial can provide access to new treatments that may not be available for you.