Ewing Sarcoma - Childhood and Adolescence

Ewing Sarcoma - Childhood and Adolescence

Ewing Sarcoma is a type of cancer that affects the bones and the surrounding soft tissues. It commonly appears in bones like the legs, hips, ribs, arms, and spine. It can also be found in soft tissues such as the thighs, hips, spine, chest, and feet.

Statistics

There is no specific data available for Ewing Sarcoma in India. In the United States, around 200 children are diagnosed with this cancer annually, accounting for only 1% of all childhood cancers.

Survival Rate

According to data from the United States, the 5-year survival rate for Ewing Sarcoma is 63%.

Risk Factors

Risk factors are conditions or elements that increase the likelihood of developing cancer, but they do not necessarily cause cancer directly. The causes of Ewing Sarcoma in children remain unclear. However, the following factors may increase the risk:

• Genetic Changes: A significant percentage of Ewing Sarcoma cells exhibit chromosomal translocations. These genetic changes are believed to contribute to the development of the cancer.
• Age: Although it can occur at any age, more than half of the patients diagnosed are between 10 and 20 years old.
• Gender: It is more common in boys than in girls.

Symptoms

• Pain, swelling, or stiffness around the bone or the surrounding area. Around 85% of children with Ewing Sarcoma experience pain. The pain may come and go but tends to worsen at night.
• A lump or mass.
• Persistent fever.
• Bone fractures without a clear cause (this may happen due to the tumor growing inside the bone).

Diagnosis

• Blood tests: CBC (Complete Blood Count), LDH (Lactate Dehydrogenase), LFT (Liver Function Test), RFT (Renal Function Test).
• X-ray: Initial imaging test to detect abnormalities in the bone.
• CT Scan: Detailed cross-sectional images to identify the tumor.
• MRI: Helps in determining the extent and size of the tumor.
• PET CT Scan: Detects cancer cells in the body.
• Bone Scan: Assesses the spread of cancer to the bones.
• Biopsy: A small piece of the suspected tumor is removed and examined under a microscope to confirm the presence of cancer cells. If the tumor is small, it may be completely removed during surgery. If it’s large or in a critical area, only a small part will be taken for biopsy.
• Bone Marrow Aspiration and Biopsy: If blood tests show changes in the number or shape of blood cells, a bone marrow biopsy or aspiration may be needed to check for cancer cells in the bone marrow.
• Immunohistochemistry and Cytogenetic Tests: Advanced testing techniques used to diagnose the tumor type and understand its genetic makeup.
• Biomarker Testing: Tests specific genes or proteins related to the cancer.

Treatment

1. Chemotherapy
Chemotherapy uses drugs to destroy cancer cells. A treatment schedule or regimen outlines how many doses of chemotherapy will be given over a specific period. For Ewing Sarcoma, chemotherapy drugs like cyclophosphamide, doxorubicin, etoposide, ifosfamide, and vincristine are commonly used. If the cancer hasn’t spread, chemotherapy is usually given every two weeks.
The side effects of chemotherapy vary depending on the drug and the individual but may include fatigue, nausea, vomiting, hair loss, infections, diarrhea, and loss of appetite. These side effects typically decrease after chemotherapy is completed.
Children receiving chemotherapy may develop neutropenia, a condition where the levels of a type of white blood cell called neutrophils drop significantly, making them more susceptible to infections. Colony-stimulating factors (CSFs) or growth factors may be given to treat this condition.
2. Surgery
The cancerous tissue and some surrounding healthy tissue (margin) are surgically removed. Surgery is usually performed after chemotherapy in cases of Ewing Sarcoma, although this can vary based on the individual. Depending on the tumor’s size and location, bone may need to be removed. Reconstruction using bone grafts or prostheses can be done if the surgery affects the function of the area. After surgery, physical therapy may help patients regain their normal activities.
3. Radiation Therapy
Radiation therapy uses high-energy radiation to kill cancer cells. External beam radiation therapy (EBRT) is delivered through a machine outside the body. Radiation is used for Ewing Sarcoma when surgery is not possible, if the tumor cannot be fully removed by surgery, or if chemotherapy was not effective.
4. Bone Marrow Transplant/Stem Cell Transplant
This treatment involves collecting specialized cells called hematopoietic stem cells from the blood and implanting them into the bone marrow. These stem cells produce blood cells like white blood cells, red blood cells, and platelets. For Ewing Sarcoma, autologous stem cell transplantation (AUTO) is used, where the patient’s own stem cells are collected and reintroduced after high-dose chemotherapy or radiation therapy destroys the cancer cells. The goal of this treatment is to help regenerate healthy bone marrow.

Ewing Sarcoma Follow-Up

After completing cancer treatment, regular follow-up tests are essential. These follow-ups help manage side effects and ensure the cancer does not return. Routine physical exams, blood tests, and imaging tests are typically included in the follow-up process.

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