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Medically reviewed by Abhijit Bhattacharyya | MD, PhD, MBA, Tufts University School of Medicine - Miami, Florida on January 5th, 2024.
Ewing sarcoma is a rare type of bone cancer that primarily affects children and young adults. This condition occurs when cancerous tumors grow in the bones or the soft tissues surrounding the bones, such as cartilage. Although Ewing's sarcoma is uncommon, it is essential to understand the types, causes, symptoms, diagnosis, and treatment options for this condition.
There are four main types of Ewing sarcoma, depending on where the tumor forms:
Bone tumor: The most common type, accounting for 87% of cases, usually affecting the thigh bones, pelvis, ribs, or upper arms.
Soft tissue tumor: This type grows in the soft tissues around the bones, such as cartilage.
Peripheral primitive neuroectodermal tumor (PNET): Found in the nerves, this type can grow in various parts of the body.
Askin tumor: A type of PNET tumor that develops in the chest wall.
All types of Ewing sarcoma have the potential to spread to other parts of the body, including the bones, bone marrow, and lungs.
The exact cause of Ewing sarcoma remains unknown. Researchers believe that changes in a cell's DNA occurring after birth are likely responsible for the development of this cancer. However, it is not linked to family history, diet, lifestyle, or environmental factors.
Common symptoms of Ewing sarcoma include:
Pain, swelling, or stiffness in the area of the tumor
A lump near the skin that feels warm and soft to the touch
Persistent low-grade fever
Limping due to leg pain
Bone pain that worsens with exercise or at night
Unexplained bone fractures
Unintentional weight loss
Fatigue without a known reason
To diagnose Ewing sarcoma and determine if the tumor has spread, doctors may perform several tests, including:
Physical exam
X-rays
Bone scan
Magnetic resonance imaging (MRI)
Computed tomography scan (CT scan)
Positron emission tomography scan (PET scan)
Biopsy
Bone marrow aspiration and biopsy
Treatment options for Ewing sarcoma depend on factors such as tumor size, location, spread, the child's overall health, and family preferences. The main treatment options include:
Chemotherapy: Usually the first step, involving medication to shrink the tumor.
Surgery: Typically follows chemotherapy, aiming to remove the tumor and prevent its spread.
Radiation therapy: Uses X-rays and other types of radiation to kill cancer cells.
The survival rate for Ewing sarcoma depends on whether the cancer has spread. According to the American Cancer Society, if the cancer hasn't spread, the 5-year survival rate is 82%. If the cancer has spread, the survival rate ranges from 39% to 71%, depending on the extent of the spread. It is important to note that these are estimates, and survival rates continue to improve with advancements in treatment.
For more information on Ewing sarcoma, visit the National Cancer Institute and the St. Jude Children's Research Hospital websites.
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